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| *APSFA's Favorites *In the News & Community - APSFA APS - *General APS - Cardiology & Pulmonology Related APS - Catastrophic Antiphospholipid Syndrome APS - Hematology & Rheumatology Related APS - Lupus Related APS - Lupus Research & Trials Information APS - Nephrology Related APS - Neurology Related APS - Opthalmology Related APS - Other APS - Pregnancy APS - Seronegative APS - SNAPS APS Support Groups & Chat Autoimmune Diseases - Other Cardiology & Pulmonology Related Information Disability Resources Doctor Information General Information Genetic Clotting Problems INR & INR Machines Loss Information Lupus Information - General Lupus Information - Journal Articles Medic Alert Information Medication Information - *General Medication Information - *Prescription Assistance Medication Information - Anticoagulants Medication Information - Antiplatelets Medication Information - Antithrombotics Medication Information - Herbals Medication Information - Other Migraine & Headache Information No Clots but Test Positive Other Medical Problems Pain & Pain Management Personal Medical Stories Pregnancy Information Professional Medical Websites Stroke & TIA Information Supports INR Greater Than 3 Thrombosis Information - *General Thrombosis Information - DVT Thrombosis Information - Other Thrombosis Information - PE | ||||||||
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| *APSFA's Favorites |
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| **APS Foundation of America, Inc | ||||||||
| Founded in June 2005, the APS Foundation of America, Inc. is dedicated to fostering and facilitating joint efforts in the areas of education, support, research, patient services and public awareness of Antiphospholipid Antibody Syndrome in an effective and ethical manner. | ||||||||
| **APS Friends & Support Forum | ||||||||
| A forum run by Heidi & Tina founders of the APS Foundation of America, Inc., a non profit organization. This forum is an information source and a friendly support group for people who have Antiphospholipid Antibody Syndrome or for anyone who's lives are touched by it. It is sometimes referred to as APS, APLS, or APLA and is known as Hughes Syndrome or "Sticky Blood" in the UK. APS is associated with recurrent clotting events including premature stroke, repeated miscarriages, phlebitis, venous thrombosis and pulmonary thromboembolism. If this disease touches your life in some way, please feel free to join in our discussions! :) We're glad to have you visit! | ||||||||
| **GoodSearch.com | ||||||||
| What if APS Foundation of America - APSFA earned a penny every time you searched the Internet? Well, now we can! GoodSearch.com is a new search engine that donates half its revenue, about a penny per search, to the charities its users designate. You use it just as you would any search engine, and it's powered by Yahoo!, so you get great results. Just go to http://www.goodsearch.com and be sure to enter APS Foundation of America - APSFA as the charity you want to support. Just 500 of us searching four times a day will raise about $7300 in a year without anyone spending a dime! And, be sure to spread the word! | ||||||||
| *Antiphospholipid Antibody Syndrome (APS) Booklet | ||||||||
| written by the APS Foundation of America, Inc. This pamphlet is a layman's terms summary of Antiphospholipid Syndrome (APS). It covers such topics as diagnosis, symptoms, treatment, and coping. It is meant for patients newly diagnosed, however would also be good for informing friends and family about your disease. | ||||||||
| *Antiphospholipid Antibody Syndrome (APS) Booklet | ||||||||
| written by the APS Foundation of America, Inc. | ||||||||
| *APS Foundation of America Brochure | ||||||||
| written by the APS Foundation of America, Inc. | ||||||||
| *Medic Alert | ||||||||
| HIGHLY recommended! This jewerly could save your life! | ||||||||
| *National Alliance for Thrombosis and Thrombophilia (NATT) | ||||||||
| The National Alliance for Thrombosis and Thrombophilia (NATT) is a nationwide, non-profit patient advocacy group representing the interests of people with blood clots and clotting disorders, including people with the APLA syndrome. NATT's mission is to address major treatment issues, such as preventing thrombosis and its complications, and reducing death and illness related to thrombosis. NATT wants patients to get involved. | ||||||||
| 2009 H1N1 Flu (referred to as "swine flu" early on) and Seasonal Flu Information for People with Inflammatory Arthritis or Rheumatic Disease | ||||||||
| Antiphospholipid Antibodies | ||||||||
| Circulation. 2005;112:e39-e44. © 2005 American Heart Association, Inc. Caron P. Misita, PharmD; Stephan Moll, MD | ||||||||
| Antiphospholipid Antibody Syndrome and Pregnancy | ||||||||
| Background: Antiphospholipid syndrome (APS) is a recently recognized autoimmune condition that may manifest with fetal loss, thrombosis, or autoimmune thrombocytopenia. Women with these clinical features should be tested for lupus anticoagulant (LAC) and anticardiolipin (aCL) antibodies; most patients with APS have both LAC and aCL immunoglobulin G (IgG) antibodies. The diagnosis of APS requires the presence of both clinical and biological features. Systemic lupus erythematosus (SLE) is a chronic systemic disease with diverse clinical and laboratory manifestations. LAC (and aCL) predisposes to clotting in vivo, predominantly by interfering with the antithrombotic role of phospholipids (PLs); therefore, it is associated with clinical thrombosis, not bleeding. The antiphospholipid (aPL) autoantibodies bind moieties on negatively charged PLs or moieties formed by the interaction of negatively charged PLs with other lipids, PLs, or proteins. aPL antibodies belong to the large family of antibodies that react with negatively charged PLs, including cardiolipin, phosphatidylglycerol, phosphatidylinositol, phosphatidylserine, phosphatidylcholine, and phosphatidic acid. Last Updated: September 4, 2005 | ||||||||
| Do you have Lupus Anticoagulants? | ||||||||
| The APSFA is currently working with a company called George King Bio-Medical, Inc and they are looking for people who are positive with the Lupus Anticoagulant (LA) to donate plasma. We are strongly urging people who qualify to take part in this opportunity as it will only make future testing more accurate! | ||||||||
| eMedicine - Antiphospholipid Antibody Syndrome : Article by Barry L Myones, MD | ||||||||
| Last Updated: October 26, 2004 Promtes an INR in the range of 2.5-3.5. | ||||||||
| eMedicine - Antiphospholipid Syndrome : Article by Steven Carsons, MD | ||||||||
| Last Updated: December 5, 2004 Based on the most recent evidence, a reasonable target for the international normalized ratio (INR) is 2.6-3 for a minimum of 6 months for a first thrombosis. Patients with recurrent thrombotic events while well maintained on the above regimen may require an INR of 3-4 and generally receive anticoagulation therapy for life. For severe or refractory cases, a combination of warfarin and aspirin may be used. | ||||||||
| FDA Vitamin K List | ||||||||
| General Management of the Patient with a Positive Antiphospholipid Antibody Test: What Evidence Is Available For You and Your Physician To Consider? | ||||||||
| Written by: Gale A McCarty, MD, FACP, FACR You are an individual and your particular case may involve some features that separate you from the study patients. What is most likely to have the right balance of "helpful vs. harmful" effects on you is the major concern of your physician to prevent the effects of aPL antibodies in contributing to blood clots and cell or organ damage in you. Let's look at the major approaches used to manage your positive aPL test. | ||||||||
| General Management of Thromboses (Blood Clot) II—What Evidence is Available For You and Your Physician To Consider? | ||||||||
| Written by: Gale McCarty, MD, FACP, FACR. As always, the physician treating the individual APS patient has to balance the wisdom of these guidelines (which are general guidelines and not mandates applicable to ALL patients) with the needs of his/her patient to find the best answer. | ||||||||
| HYDROXYCHLOROQUINE - EVERYTHING OLD IS NEW AGAIN! | ||||||||
| By: Gale McCarty, MD, FACR, FACP. Hydroxychloroquine (HCQ, or its trade name-Plaquenil) has a long and honored history of use in systemic lupus erythematosus (SLE) as a general medication to decrease activity of the immune system and decrease symptoms. For years it has been approved for use by the FDA for lupus and rheumatoid arthritis, and has been used most frequently for skin and joint manifestations. It is considered a mainstay of therapy for any patient with SLE by many lupus experts and rheumatologists. It has many mechanisms of action, some related to decrease in the activity of the immune system, and some related to effects on blood clotting mechanisms. HCQ belongs to the class of drugs call anti-malarials, which includes Chloroquine and Atabrine. (This does not mean that anyone thinks that SLE or APS is caused by the agent that causes malaria-like most discoveries in medicine, it was the chance observation that patients with some autoimmune diseases who got anti-malarial drugs to prevent malaria when traveling to likely areas of infection noted their symptoms improved on HCQ). One of the most complete and excellent reviews of all the literature on the anti-malarials to which all patients and their physicians are directed is Dr. Dan Wallace's Chapter 59 in the Wallace-Hahn Dubois' Lupus Erythematosus textbook. Another excellent review on APS therapy in general has been published by Dr. Robert Roubey. | ||||||||
| I Have the Lupus Anticoagulant, But I Don't Have Lupus? | ||||||||
| By: Thomas L. Ortel, MD, PhD "...although the 'lupus' anticoagulant was first described in several patients with lupus, most patients with lupus anticoagulants actually don't have any of the other clinical manifestations of lupus." | ||||||||
| Influenza Vaccination in Patients with Antiphospholipid Antibodies | ||||||||
| Written by: Thomas L. Ortel, MD, PhD | ||||||||
| Is Antiphospholipid Antibody Syndrome Hereditary? Should My Children Get Tested? | ||||||||
| Written by: Thomas L. Ortel, MD, PhD | ||||||||
| Listen to the Patient — Anticoagulation Is Critical in the Antiphospholipid (Hughes) Syndrome | ||||||||
| © 2003. The Journal of Rheumatology Publishing Company Limited. Promotes an INR of greater than 3.0. | ||||||||
| Lupus Anticoagulant: Testing While on Anticoagulant Therapy: Can It Be Done? | ||||||||
| Written by: Thomas L Ortel, MD, PhD. In conclusion, it is optimal to test for a lupus anticoagulant when the patient is on no anticoagulant therapy. All of the test results can be interpreted more easily in that setting. Sometimes this can be difficult to arrange, however, and testing needs to be performed while the patient is still taking anticoagulants. In this situation, the doctor needs to work carefully with the laboratory, to understand how the tests are being performed and to make sure that the results are interpreted correctly. | ||||||||
| Medical Progress- Antiphospholipid Antibody Syndrome | ||||||||
| PDF File from the New England Journal of Medicine | ||||||||
| Migraine, memory loss, and "multiple sclerosis ". Neurological features of the antiphospholipid (Hughes') syndrome | ||||||||
| Postgraduate Medical Journal 2003;79:81-83 © 2003 Fellowship of Postgraduate Medicine. Promotes an INR of greater than 3.0. | ||||||||
| Monitoring Warfarin Therapy in Patients with Lupus Anticoagulants | ||||||||
| Recommended therapeutic international normalized ratios (INRs) for oral anticoagulation in patients with lupus anticoagulants who sustain a thromboembolic event are controversial. Patients with lupus anticoagulants often have a prolonged prothrombin time, which may complicate management of anticoagulant therapy. | ||||||||
| New subsets of the antiphospholipid syndrome in 2006: "PRE-APS" (probable APS) and microangiopathic antiphospholipid syndromes ("MAPS"). | ||||||||
| Autoimmun Rev, December 1, 2006; 6(2): 76-80. The concept of "probable" antiphospholipid syndrome (APS) is almost identical with several conditions which may presage the development of the APS with its major complications of large vessel thromboses resulting in deep vein occlusions in the lower limbs (DVT) particularly and strokes. These conditions comprising livedo reticularis, chorea, thrombocytopenia, fetal loss and valve lesions. These conditions, comprising livedo reticularis, chorea, thrombocytopenia, fetal loss and valve lesions may be followed, often years later by diagnosable APS. The issue whether these patients should be more aggressively treated on presentation in order to prevent the thrombotic complications. A new subset of the APS is proposed viz. microangiopathic antiphospholipid syndrome ("MAPS") comprising those patients presenting with thrombotic microangiopathy and demonstrable antiphospholipid antibodies who may share common although not identical provoking factors (e.g. infections, drugs), clinical manifestations and haematological manifestations (severe thrombocytopenia, hemolytic anaemia) and treatments viz. plasma exchange. Patients without large vessel occlusions may be included in the MAPS subset. These conditions include thrombotic thrombocytopenic purpura (TTP), hemolytic-uremic syndrome (HUS), and the HELLP syndrome. Patients with catastrophic antiphospholipid syndrome (CAPS) who do not demonstrate large vessel occlusions also fall into this group. Disseminated intravascular coagulation (DIC) has also been reported with demonstrable antiphospholipid antibodies and also manifests severe thrombocytopenia and small vessel occlusions. It may cause problems in differential diagnosis. | ||||||||
| Predicting Thrombosis Risk in Individuals with Antiphospholipid Antibodies | ||||||||
| Written by: Thomas L Ortel, MD, PhD "...several studies have shown that the presence of a lupus anticoagulant in the blood is associated with a higher risk for a clot than the presence of an anticardiolipin antibody." | ||||||||
| Quarterly Newsletter "Antiphospho....What?" | ||||||||
| Volume 10 - Summer/Fall 2008. Written by the APS Foundation of America, Inc. | ||||||||
| Quarterly Newsletter "Antiphospho....What?" | ||||||||
| Volume 16 - Winter/Spring 2010. written by the APS Foundation of America, Inc. | ||||||||
| Rare Diseases Clinical Research Network | ||||||||
| The Rare Diseases Clinical Research Network was created to facilitate collaboration among experts in many different types of rare diseases. Our goal is to contribute to the research and treatment of rare diseases by working together to identify biomarkers for disease risk, disease severity and activity, and clinical outcome, while also encouraging development of new approaches to diagnosis, prevention, and treatment. | ||||||||
| Rare Thrombotic Diseases Consortium (RTDC) | ||||||||
| The Rare Thrombotic Diseases Consortium (RTDC) is an integrated group of academic medical centers, patient support organizations, and clinical research resources dedicated to conducting clinical research in different forms of and improving the care of patients with thrombotic diseases. Funded by the National Institutes of Health (NIH), the RTDC is part of the Rare Diseases Clinical Research Network. The operations of the RTDC are directed from Duke University. Other primary RTDC study sites include the University of North Carolina, University of Wisconsin, Centers for Disease Control and Prevention, and the Mayo Clinic. | ||||||||
| Sapporo Criteria for Diagnosing APLA Syndrome | ||||||||
| SERONEGATIVE ANTIPHOSPHOLIPID ANTIBODY SYNDROME (SNAPS)…AND SNAPPING TO IT!! | ||||||||
| By: Gale McCarty, MD, FACR, FACP. "You don't have the syndrome because your tests are low level or negative…" or "You have livedo, a heart valve problem, and thrombocytopenia, but these aren't listed as criteria for diagnosis" are comments made frequently by healthcare providers from many specialties to patients with clinical features suggesting the Antiphospholipid Antibody Syndrome (APS). | ||||||||
| Surgical Management of the Primary Dental Patient on Warfarin | ||||||||
| Written to inform Dentists about treating Warfarin Patients | ||||||||
| The 13th International Congress on Antiphospholipid Antibodies (APLA 2010) | ||||||||
| Written by: Silvia Pierangeli | ||||||||
| Thrombosis and the Antiphospholipid Syndrome | ||||||||
| Hematology 2005 © 2005 The American Society of Hematology. Summary: Even with the most complete datasets, it is still important for the physician to develop a therapeutic plan appropriate for the individual patient, based on clinical presentation, co-morbid conditions, and other variables. With uncommon disorders and limited datasets, such as with the antiphospholipid syndrome, decision-making becomes even more difficult. Table 3 presents a strategy that the author uses when evaluating and developing a treatment plan for a patient with antiphospholipid syndrome and thrombosis, based on the available studies summarized in this article. Critical areas for future research include identifying which patients with antiphospholipid antibodies are at highest risk for thrombotic complications, developing new antithrombotic agents that are effective and safe, and investigating novel approaches to eliminate the autoantibody and, hopefully, the increased prothrombotic state. | ||||||||
| Thrombosis Interest Group of Canada | ||||||||
| The Thrombosis Interest Group of Canada consists of a group of 40 specialists in fields related to thrombosis who collaborate to write evidence-based or consensus-based clinical guides on the investigation, management, and diagnosis of thrombotic disorders. | ||||||||
| Warfarin in Antiphospholipid Syndrome — Time to Explore New Horizons | ||||||||
| The Journal of Rheumatology Feb. 2005 Promotes an INR greater than 3.0. | ||||||||
| What is "Micro-Clotting"? | ||||||||
| By: Thomas L. Ortel, MD, PhD Simply put, micro-clotting, better referred to as "microvascular thrombosis"' describes blood clotting that is occurring in some of the smallest blood vessels in the body. | ||||||||
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| *In the News & Community - APSFA |
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| 2nd Annual Venous Disease Coalition Annual Meeting | ||||||||
| Surgeon General Announces Call to Action at VDC Meeting Members of the Venous Disease Coalition (VDC) and the Office of the Surgeon General came together at the VDC Annual Meeting in Washington D.C. on Monday, September 15th. Acting Surgeon General Rear Admiral Steven K. Galson issued "The Surgeon General's Call to Action for the Preventionof Deep Vein Thrombosis and Pulmonary Embolism," which combined affect hundreds of thousands of Americans each year. Dr. Gale McCarty & Dr. Tom Ortel represented the APSFA at this annual meeting. | ||||||||
| American Stroke Association: Stroke Connection Magazine, November/December 2006, Page 44 | ||||||||
| Antiphospho....what?! | ||||||||
| The APS Foundation of America, Inc. is the only United States nonprofit health agency dedicated to bringing national awareness to Antiphospholipid Antibody Syndrome (APS), the major cause of multiple miscarriages, thrombosis, young strokes and heart attacks. We are a volunteer run, community based 501(c)3 non-profit Public Charity organization and is dedicated to fostering and facilitating joint efforts in the areas of education, support, public awareness, research and patient services. | ||||||||
| Antiphospholipid Antibody Syndrome (APS) Sufferers Start Non-profit Health Agency: Announcing the APS Foundation of America official website www.apsfa.org | ||||||||
| Press Release - October 27, 2007 on PRWEB | ||||||||
| Antiphospholipid Antibody Syndrome Awareness Month | ||||||||
| Michigan Proclamation for 2007 | ||||||||
| APS Awareness PSA | ||||||||
| APS Awareness PSA for WFLS | ||||||||
| PSA airing on 93.3 WFLS- Real Country Variety, Fredericksburg, VA | ||||||||
| APS Awareness Starts Here | ||||||||
| Written by: Charles Strickler. What is APS? How do we increase awareness of APS? What research needs to be done to improve treatment options? These were some of the many questions discussed at the awareness and fundraising dinner that was hosted by me, my wife, Mary Strickler and our co-hosts, David and Kim Penberthy. We held the dinner May 17th, and it seemed more than appropriate to have such an event just prior to APS Awareness Month in June! | ||||||||
| APS FOUNDATION OF AMERICA, INC. JOINS WITH NORD TO CELEBRATE THE 25th ANNIVERSARY OF THE ORPHAN DRUG ACT | ||||||||
| APSFA Press Release - January 2008 | ||||||||
| APS in the Community | ||||||||
| On April 22nd the APSFA made an appearance at the annual March of Dimes WalkAmerica event in Springfield, Virginia. | ||||||||
| APS is a snake in the grass and can bite or kill without warning | ||||||||
| APSFA Press Release - Week 4 - 2007 | ||||||||
| APS Linked to Birth Difficulties | ||||||||
| APSFA Press Release - Week 2 - 2007 | ||||||||
| APSFA To the Rescue | ||||||||
| Written by: Seren Estrada Thanks to the support of the APSFA, I expect to continue my education with the help of the DSSO. It also goes to show that the APS Foundation of America, Inc is truly dedicated to advocating for people with APS. | ||||||||
| Eau Claire, Leader-Telegram, November 29, 2006, Page 1C | ||||||||
| Friends to remember Tom Sparks | ||||||||
| Bill Sparks said in a statement that doctors have determined that Tom's stroke was probably caused by a condition called antiphospholipid antibody syndrome (APS), a disease that causes unnatural clotting of the blood. In lieu of flowers, the family has asked that donations in Tom's memory be sent to the APS Foundation of America, the only nonprofit organization in the country dedicated to bringing awareness to APS. | ||||||||
| Grand Rounds at Marshfield Clinic | ||||||||
| written by Tina Pohlman | ||||||||
| June is APS Awareness Month PSA | ||||||||
| June is Antiphospholipid Antibody Syndrome (APS) Awareness month. | ||||||||
| June is APS Awareness Month PSA #1 (2009) | ||||||||
| (c) APS Foundation of America, Inc. Special thanks to Jeff Cecil of JMC Creative for producing the audio. | ||||||||
| June is APS Awareness Month PSA #2 (2009) | ||||||||
| (c) APS Foundation of America, Inc. Special thanks to Jeff Cecil of JMC Creative for producing the audio. | ||||||||
| June is APS Awareness Month PSA #3 (2009) | ||||||||
| (c) APS Foundation of America, Inc. Special thanks to Jeff Cecil of JMC Creative for producing the audio. | ||||||||
| June is APS Awareness Month PSA #4 (2009) | ||||||||
| (c) APS Foundation of America, Inc. Special thanks to Jeff Cecil of JMC Creative for producing the audio. | ||||||||
| June is APS Awareness Month ~ Get In the Flow! | ||||||||
| Written by: Tina Pohlman | ||||||||
| June is APS Awareness Month: Get in the Flow! | ||||||||
| The APS Foundation of America, Inc. (APSFA) has declared June as National Antiphospholipid Antibody (APS) Awareness Month. We are educating the public and medical community about this disorder, urging people to Get in the Flow! | ||||||||
| JUNE IS APS AWARENESS MONTH: GET IN THE FLOW! | ||||||||
| APSFA Press Release - Week 1 - 2007 | ||||||||
| JUNE IS APS AWARENESS MONTH: GET IN THE FLOW! | ||||||||
| The APS Foundation of America, Inc. (APSFA) has declared June as National Antiphospholipid Antibody (APS) Awareness Month. We are educating the public and medical community about this disorder, urging people to Get in the Flow! 2009 | ||||||||
| Lupus Foundation of America: Pacific Northwest Chapter: In the Lupe! December 2006, Page 5 | ||||||||
| Lupus Foundation of Ontario: Lupus Talk, October 2006 to December 2006, page 5 | ||||||||
| MO-KAN Spreads APS Awareness | ||||||||
| Written by Dana Stuart. "Mo-Kan" is a phrase commonly used in the Missouri-Kansas region to describe various missions or goals that Missouri and Kansas businesses and residents CAN accomplish together. Frequently used for public service announcements, advertisements, and other miscellaneous uses, "Mo-Kan" has become the unofficial slogan for several alliances, which have formed for some very worthy causes. Now "Mo-Kan" spreads APS Awareness! | ||||||||
| One Fine Day | ||||||||
| Written by: Dana Stuart | ||||||||
| Our Journey with Mystery Diagnosis | ||||||||
| written by Michelle LaRue | ||||||||
| Proclamation 2007 - State of CT | ||||||||
| Thank you for your support! | ||||||||
| Proclamation 2007 - State of KY | ||||||||
| Thank you for your support! | ||||||||
| Proclamation 2007 - State of MO | ||||||||
| Thank you for your support! | ||||||||
| Proclamation 2007 - State of OH | ||||||||
| Thank you for your support! | ||||||||
| Proclamation 2007 - State of PA | ||||||||
| Thank you for your support! | ||||||||
| Proclamation 2007 - State of WI | ||||||||
| Thank you for your support! | ||||||||
| Quarterly Newsletter "Antiphospho....What?" | ||||||||
| Volume 3 - Fall 2006. written by the APS Foundation of America, Inc. | ||||||||
| Quarterly Newsletter "Antiphospho....What?" | ||||||||
| Volume 2 - Summer 2006. written by the APS Foundation of America, Inc. | ||||||||
| Quarterly Newsletter "Antiphospho....What?" | ||||||||
| Volume 1 - Spring 2006. written by the APS Foundation of America, Inc. | ||||||||
| Quarterly Newsletter "Antiphospho....What?" | ||||||||
| Volume 4 - Fall 2006. written by the APS Foundation of America, Inc. | ||||||||
| Quarterly Newsletter "Antiphospho....What?" | ||||||||
| Volume 5 - Spring 2007. written by the APS Foundation of America, Inc. | ||||||||
| Quarterly Newsletter "Antiphospho....What?" | ||||||||
| Volume 6 - Summer 2007. written by the APS Foundation of America, Inc. | ||||||||
| Quarterly Newsletter "Antiphospho....What?" | ||||||||
| Volume 7 - Fall 2007. written by the APS Foundation of America, Inc. | ||||||||
| Quarterly Newsletter "Antiphospho....What?" | ||||||||
| Volume 8 - Winter/Spring 2008. written by the APS Foundation of America, Inc. | ||||||||
| Quarterly Newsletter "Antiphospho....What?" | ||||||||
| Volume 9 - Spring/Summer 2008. Written by the APS Foundation of America, Inc. | ||||||||
| Quarterly Newsletter "Antiphospho....What?" | ||||||||
| Volume 12 - Winter/Spring 2009. written by the APS Foundation of America, Inc. | ||||||||
| Quarterly Newsletter "Antiphospho....What?" | ||||||||
| Volume 11 - Fall/Winter 2008. written by the APS Foundation of America, Inc. | ||||||||
| Quarterly Newsletter "Antiphospho....What?" | ||||||||
| Volume 13 - Spring/Summer 2009 written by the APS Foundation of America, Inc. | ||||||||
| Quarterly Newsletter "Antiphospho....What?" | ||||||||
| Volume 10 - Summer / Fall 2008. written by the APS Foundation of America, Inc. | ||||||||
| Quarterly Newsletter "Antiphospho....What?" | ||||||||
| Volume 13 - Spring / Summer 2009. written by the APS Foundation of America, Inc. | ||||||||
| Quarterly Newsletter "Antiphospho....What?" | ||||||||
| Volume 14 - Summer / Fall 2009. written by the APS Foundation of America, Inc. | ||||||||
| Rare condition often overlooked | ||||||||
| By Shannon Farr, Saturday, June 30, 2007 | ||||||||
| Rare disorder's diagnosis draws media attention | ||||||||
| Published: Sunday, November 20, 2005 12:03 AM CST. A Ventura teen's struggle to obtain a diagnosis for a relatively rare auto-immune disorder will be featured on the Discovery Health Channel. Angie Abbas, 18, who has Antiphospholipid Antibody Syndrome (APS), will be featured in a segment of "Mystery Diagnosis" in early 2006. The date has not been determined. Angie's diagnosis was difficult because APS mimics other disorders, LaRue said. In addition, Angie's primary symptom, a severe twitching of the head and neck, is not typical. In the meantime, producers of "Mystery Diagnosis" had learned about Angie through the APS Foundation of America, a relatively new organization in which Angie and her mother had become active. | ||||||||
| Sisters' ailment identified | ||||||||
| 07/23/2007 - By Christy Murdoch, For the Herald-Standard | ||||||||
| Social Networking for APSFA | ||||||||
| Strokes and heart attacks could be caused by APS | ||||||||
| APSFA Press Release - Week 3 - 2007 | ||||||||
| Thrombosis Education Days ~ MI & Chicago | ||||||||
| written by Heidi Ponagai. The National Alliance of Thrombosis and Thrombophilia (NATT) and the MSU Center for Bleeding and Clotting Disorders held an education day on March 24th in Lansing, Michigan for people who have had blood clots, who have clotting disorders, and their families and friends. We were fortunate enough to get a booth at the seminar to display and distribute APSFA brochures and booklets to people. | ||||||||
| Ventura teen on 'Mystery Diagnosis' on March 6 | ||||||||
| VENTURA — A program featuring a Ventura teen's struggle to obtain a diagnosis for a relatively rare auto-immune disorder will be aired March 6 on the Discovery Health Channel, the show's producers have announced. Angie Abbas, 18, who has Antiphospholipid Antibody Syndrome (APS), will be featured at 9 p.m. Central Standard Time on March 6. "Mystery Diagnosis" is a series that tells the stories of how real people have obtained difficult diagnoses. It is produced through True Entertainment LLC of New York City. The program was filmed in October at a Minneapolis production studio, Mercy Medical Center-North Iowa in Mason City and Angie's home in rural Ventura. The Discovery Health Channel is Channel 203 on Mediacom's digital-plus package or Channel 93 through CL Tel. | ||||||||
| WFLS 93.3 APSFA Air Check - Sample 1 | ||||||||
| These are air checks that are currently hitting the DC Metro Area. About 1 million listeners at any given time. | ||||||||
| WFLS 93.3 APSFA Air Check - Sample 2 | ||||||||
| These are air checks that are currently hitting the DC Metro Area. About 1 million listeners at any given time. | ||||||||
| WFLS 93.3 APSFA Air Check - Sample 3 | ||||||||
| These are air checks that are currently hitting the DC Metro Area. About 1 million listeners at any given time. | ||||||||
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| APS - *General |
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| *Antiphospholipid Antibody Syndrome | ||||||||
| Last Updated: October 26, 2004 Author: Barry L Myones, MD, Director of Research, Pediatric Rheumatology Center, Texas Children's Hospital at Houston; Associate Professor, Departments of Pediatrics & Immunology, Pediatric Rheumatology Section, Baylor College of Medicine Coauthor(s): Deborah McCurdy, MD, Director of Rheumatology, Department of Pediatric Rheumatology, Children's Hospital of Orange County Barry L Myones, MD, is a member of the following medical societies: American Academy of Pediatrics, American Association for the Advancement of Science, American Association of Immunologists, American College of Rheumatology, American Heart Association, American Society for Microbiology, Clinical Immunology Society, and Texas Medical Association Editor(s): Terry Chin, MD, Allergy/Immunology/Pulmonology; Co-Director Cystic Fibrosis and Home Ven, Associate Professor of Pediatrics, Department of Pediatrics, Loma Linda University and Children's Hospital; Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; David D Sherry, MD, Professor of Pediatrics, University of Pennsylvania; Director of Clinical Rheumatology, Division of Rheumatology, Children's Hospital of Philadelphia; Daniel Rauch, MD, Director, Pediatric Hospitalist Program, Associate Professor, Department of Pediatrics, New York University School of Medicine; and Norman T Ilowite, MD, Chief, Division of Rheumatology, Schneider Children's Hospital; Professor, Department of Pediatrics, Albert Einstein College of Medicine | ||||||||
| *Antiphospholipid Antibody Syndrome (APS) Booklet | ||||||||
| written by the APS Foundation of America, Inc. This pamphlet is a layman's terms summary of Antiphospholipid Syndrome (APS). It covers such topics as diagnosis, symptoms, treatment, and coping. It is meant for patients newly diagnosed, however would also be good for informing friends and family about your disease. | ||||||||
| *Antiphospholipid Antibody Syndrome (APS) Booklet | ||||||||
| written by the APS Foundation of America, Inc. | ||||||||
| *Antiphospholipid Syndrome (APS) | ||||||||
| Written by: Jan M. Pankey, M.D. Children's Hospital & Research Center Oakland, California for the National Alliance for Thrombosis and Thrombophilia (NATT) | ||||||||
| *APS & Surgery Brochure | ||||||||
| written by the Hospital for Special Surgery | ||||||||
| *APS & You | ||||||||
| written by the APS Foundation of America, Inc. This pamphlet is a layman's terms summary of Antiphospholipid Syndrome (APS). It covers such topics as diagnosis, symptoms, treatment, and coping. It is meant for patients newly diagnosed, however would also be good for informing friends and family about your disease. | ||||||||
| *APS Information Brochure | ||||||||
| written by the Hospital for Special Surgery. | ||||||||
| *Current News Articles Dealing with APS & Related Problems | ||||||||
| This link contains full news articles and many up to date medical journal abstracts dealing with APS and its related diseases. Free Registration is required. | ||||||||
| *The Antiphospholipid Syndrome | ||||||||
| written by Dr. Yaniv & Professor Yehuda Shoenfeld. Copyright 2004. Redistributed with permission from authors. | ||||||||
| *What is Antiphospholipid Antibody Syndrome? | ||||||||
| written by the Rare Thrombotic Disease Consortium. Redistributed with permission from the authors. | ||||||||
| *Women & APS | ||||||||
| written by the APS Foundation of America, Inc. | ||||||||
| A Patients Guide to APS | ||||||||
| Doctor Hughes Book on a website | ||||||||
| About thrombosis: thrombophilia: acquired thrombophilia: APS | ||||||||
| Another article that supports an INR of 3.0 to 4.0 for APS patients. | ||||||||
| American Venous Forum - Patients Section | ||||||||
| Founded in 1988, the American Venous Forum provides a serious academic colloquium to physicians interested in the research, education, and clinical investigation in the field of venous diseases. The Forum membership includes more than 225 board-certified vascular surgeons who have an accomplished record of interest and contribution to the management of venous disease. The mission of the American Venous Forum is to improve the care of patients with venous and lymphatic disorders by providing a forum dedicated to education and to the exchange of information concerning basic and clinical research pertaining to the venous and lymphatic systems. In past years, the Forum has dedicated itself to academic pursuits through vigorous educational meetings throughout the country. Today, the Forum is a diverse organization that includes a directory of experienced investigators and clinicians, several of whom can also speak or assist in research on a variety of venous-related topics. The Forum also offers guidelines and protocols for the development of research and clinical trials. With the guidance of the AVF governing officers, the AVF will continue to bring medical professionals and patients the latest venous health information. The AVF By-Laws include more information on our objectives, committees, meetings, and dues. | ||||||||
| Anti-phospholipid antibody syndrome | ||||||||
| The MayoFoundation for Medical Education and Research provides the following web link addressing antiphospholipid syndrome. | ||||||||
| Anticardiolipin Test and the Antiphospholipid (Hughes) Syndrome: 20 Years and Counting! | ||||||||
| © 2004. The Journal of Rheumatology Publishing Company Limited. | ||||||||
| ANTIPHOSPHOLIPID ANTIBODIES | ||||||||
| by Dr. Ron Ascherson | ||||||||
| Antiphospholipid Antibody Syndrome | ||||||||
| Antiphospholipid syndrome (APS) is characterized by the following: venous or arterial thrombosis--a condition where clots, called thrombi, form in the blood vessels; recurrent miscarriages--the repeated loss of the fetus in pregnancies; and thrombocytopenia--a low number of blood platelets that can lead to bleeding, seen as bruising and tiny red dots on the skin. Medical College of Wisconsin. | ||||||||
| Antiphospholipid Antibody Syndrome | ||||||||
| Intelihealth Website | ||||||||
| ANTIPHOSPHOLIPID ANTIBODY SYNDROME | ||||||||
| (APS) is a term used to describe the association between recurrent clinical events such as thrombosis (arterial or venous), thrombocytopenia, or fetal loss and the presence of a persistent antiphospholipid antibody 1-3. Other clinical conditions associated with the syndrome include stroke, transient ischemic attack, livedo reticularis, migraine, epilepsy, and heart valve disease4. The syndrome is termed "primary" if there is no accompanying autoimmune disease and "secondary" if the patient also has systemic lupus erythematosus (SLE) or an autoimmune disorder1,2,5. Certain infectious diseases and drugs may also result in the formation of antiphospholipid antibodies which do not appear to be associated with clinical complications and do not require therapy1,3,5. | ||||||||
| Antiphospholipid Antibody Syndrome | ||||||||
| Indian Pediatrics 2001; 38: 1413-1416 Promotes an INR greater than 3. | ||||||||
| Antiphospholipid Antibody Syndrome (APS) | ||||||||
| Neuroland | ||||||||
| Antiphospholipid Antibody Syndromes (APS) | ||||||||
| from the Rare Thrombotic Diseases Consortium | ||||||||
| Antiphospholipid or Hughes' syndrome | ||||||||
| Supports an INR of 3.0 to 4.0 for APS patients. | ||||||||
| Antiphospholipid Syndrome | ||||||||
| Antiphospholipid syndrome (APS) is characterized by the following: venous or arterial thrombosis--a condition where clots, called thrombi, form in the blood vessels; recurrent miscarriages--the repeated loss of the fetus in pregnancies; and thrombocytopenia--a low number of blood platelets that can lead to bleeding, seen as bruising and tiny red dots on the skin. | ||||||||
| Antiphospholipid Syndrome | ||||||||
| Information provided by the UMHS Hemophilia and Coagulation Disorders Program, February 2003 | ||||||||
| Antiphospholipid syndrome | ||||||||
| The antiphospholipid syndrome (APS) is characterized by venous and/or arterial thrombosis, recurrent pregnancy loss and the presence of antiphospholipid antibodies. The antiphospholipid antibodies (anticardiolipin, anti-bêta2GPI antibodies, lupus anticoagulant) interacting with various coagulation proteins, platelets or endothelial cells may contribute to disease pathogenesis. Incidence remains unknown, however the reported prevalence of antiphospholipid antibodies in the general population is low (1-4.5%) and increases with age. The main clinical manifestations associated with APS are thromboses, pregnancy morbidity, thrombocytopenia, neurological symptoms, livedo reticularis, hemolytic anemia. The antiphospholipid antibodies have been detected in approximately 1/3 of the patients with systemic lupus erythematosus (SLE). High anticardiolipin antibodies titers, lupus anticoagulant and especially anti-bêta2GPI antibodies are important predictors of APS clinical manifestations in SLE patients. The management of thrombosis includes long-term, high-intensity warfarin therapy [International Normalized Ratio (INR superior or equal to 3)]. For pregnancy morbidity the recommended therapy is low-dose aspirin (80 mg/day) plus subcutaneous unfractionated heparin or low-molecular-weight heparin. | ||||||||
| Antiphospholipid Syndrome | ||||||||
| Last modified Monday, August 10, 2007 | ||||||||
| Antiphospholipid Syndrome | ||||||||
| *Summary Points Antiphospholipid syndrome is characterized by the presence of venous and/or arterial thrombosis and/or pregnancy morbidity and the presence of antiphospholipid antibodies. Long-term anticoagulation is recommended for antiphospholipid syndrome patients with recurrent vascular events. For antiphospholipid syndrome patients with recurrent pregnancy events, aspirin plus heparin is recommended during pregnacy. Promotes an INR of >3. | ||||||||
| Antiphospholipid Syndrome | ||||||||
| Last Updated: August 10, 2007 | ||||||||
| Antiphospholipid Syndrome | ||||||||
| Copyright © 1996-2005 C. Stephen Foster M.D. All Rights Reserved. | ||||||||
| Antiphospholipid Syndrome | ||||||||
| Virtual Medical Centre. Modified: 14/7/2006 | ||||||||
| Antiphospholipid syndrome | ||||||||
| An. Bras. Dermatol. vol.80 no.3 Rio de Janeiro May/June 2005. Antiphospholipid syndrome is an acquired multisystem disorder characterized by recurrent thromboses in the arterial system, venous system, or both. Antiphospholipid syndrome is classified into 2 groups: primary and secondary. Secondary antiphospholipid syndrome is often associated with systemic lupus erythematosus and less frequently with infections, drugs and other diseases. Serologic markers are antiphospholipid antibodies, lupus anticoagulant and anticardiolipin. The primary diagnostic criteria include arterial thrombosis or venous thrombosis and recurrent fetal loss. About 41% of patients with lupus anticoagulant have skin lesions as the first sign of antiphospholipid syndrome. Cutaneous manifestations include livedo reticularis, cutaneous ulceration and livedo vasculitis. The mainstays of prophylaxis and treatment of thrombosis are anticoagulant and antiplatelet agents. | ||||||||
| Antiphospholipid Syndrome (APS) Education | ||||||||
| Michael D. Lockshin, MD Attending Rheumatologist, Hospital for Special Surgery Professor of Medicine, Weill Cornell Medical College Director, Barbara Volcker Center for Women and Rheumatic Disease. In this presentation, Dr. Lockshin provides an explanation of antiphospholipid syndrome (APS) and how it can be treated. He then answers questions submitted by patients in attendance. | ||||||||
| Antiphospholipid Syndrome - Making the Diagnosis | ||||||||
| SJS - September 2003 Levine JS, Branch DW, Rauch J. The antiphospholipid syndrome. N Engl J Med. 2002;346(10):752-63. | ||||||||
| Antiphospholipid Syndrome from eMedicine | ||||||||
| Last Updated: December 5, 2004 Author: Steven Carsons, MD, Chief, Division of Rheumatology, Allergy, and Immunology, Professor of Medicine, Department of Internal Medicine, Winthrop University Hospital, State University of New York at Stony Brook | ||||||||
| Antiphospholipid syndrome: an overview | ||||||||
| CMAJ • June 24, 2003; 168 (13) © 2003 Canadian Medical Association or its licensors | ||||||||
| APS Article on Medicine Net | ||||||||
| Medical Author: William C. Shiel Jr., MD, FACP, FACR Last Editorial Review: 9/18/2005 | ||||||||
| APS Foundation of South Africa | ||||||||
| APSSA is a foundation formed to promote much needed awareness of the Antiphospholipid Syndrome (APS) in South Africa. | ||||||||
| Euro-Phospholipid on Line | ||||||||
| Official Web-site of the "European Forum on Antiphospholipid Antibodies" | ||||||||
| Hughes' Syndrome | ||||||||
| In 1983 and during the following two years, the Lupus Research Unit at St Thomas published a number of papers showing that certain blood proteins (antiphospholipid antibodies) were associated with a syndrome of clotting (thrombosis), recurrent miscarriages and brain disease. Between 1983 and 1985, a comprehensive clinical/laboratory profile was presented showing, for the first time, a wide spectrum of clinical features including the association with artery thrombosis (including major organs such as kidney and liver), brain disease (strokes and other features), skin rashes, low platelet counts, epilepsy and migraine. | ||||||||
| Management of Antiphospholipid Antibody Syndrome | ||||||||
| Lim W, Crowther MA, Eikelboom JW. Management of antiphospholipid antibody syndrome. A systematic review. JAMA 2006; 295:1050–1057 | ||||||||
| Managing antiphospholipid syndrome | ||||||||
| Copyright Springhouse Corporation Mar 2004 | ||||||||
| Medical Progress- Antiphospholipid Antibody Syndrome | ||||||||
| PDF File from the New England Journal of Medicine | ||||||||
| New Treatments For Lupus Anticoagulants | ||||||||
| NORD - National Organization for Rare Disorders, Inc. | ||||||||
| Copyright 1994, 1995, 1996, 2001, 2002 | ||||||||
| Official Web-site of the "European Forum on Antiphospholipid Antibodies" | ||||||||
| Old-fashioned detective work | ||||||||
| August 20, 2005 | ||||||||
| Pathogenesis of the Antiphospholipid Syndrome | ||||||||
| SINDROME da ANTICORPI ANTIFOSFOLIPIDI | ||||||||
| This page is in Italian. | ||||||||
| The "primary" antiphospholipid syndrome: major clinical and serological features. | ||||||||
| Medicine (Baltimore). 1989 Nov;68(6):366-74. | ||||||||
| The Antiphospholipid Story | ||||||||
| © 2003. The Journal of Rheumatology Publishing Company Limited. | ||||||||
| The Antiphospholipid Story | ||||||||
| Before the concept of an antiphospholipid syndrome originated, lupus patients with venous occlusions and particularly those with arterial occlusions were treated mainly with corticosteroids and immunosuppressives. In addition, patients with primary APS were often diagnosed as lupus and met classification criteria for this disease. This could have been considered reasonable were it not for the unnecessary steroid treatment they received instead of merely anticoagulant and/or platelet antiaggregant treatment. DONATO ALARCóN-SEGOVIA, MD, MS, PhD. J Rheumatol. VOLUME 30: NO. 9 SEPTEMBER 2003. | ||||||||
| The Antiphospholipid Syndrome | ||||||||
| Most patients with venous or arterial thrombosis and APS do well with conventional warfarin treatment (target INR 2.0 - 3.0). It is recommended that patients with recurrent thrombosis despite conventional doses of warfarin should maintain an INR of 3.0 - 4.0. This recommendation is based on one descriptive study and requires confirmation by randomised trials. The benefit of adding aspirin in arterial disease is not clear, and is likely to increase the risk of bleeding. | ||||||||
| The Antiphospholipid Syndrome | ||||||||
| US Pharm. 2008;33(1):HS-23-HS-30. Conclusion: APS is an autoimmune disorder that presents unique challenges in diagnosis and treatment. The revised diagnosis can be simply stated as the presence of one clinical and one laboratory criteria. In practice, the heterogeneous presentation of both clinical and laboratory findings complicates diagnosis. This disorder should always be included in differential diagnosis of persons who have coagulation defects, evidence of vascular thrombosis, and/or history of recurrent miscarriages or fetal losses. Positive laboratory testing should always be confirmed at least 12 weeks apart to verify persistence. The heterogeneous presentation of APS also makes treatment challenging, and, in particular, research clarifying optimal therapy remains lacking. Currently, the mainstay of treatment is anticoagulation in those persons who develop an acute thrombotic event. Although indefinite duration of anticoagulation therapy is recommended, the decision to administer long-term anticoagulation certainly requires judicious clinical evaluation and risk assessment, given the potential hemorrhagic complications of anticoagulation therapy. | ||||||||
| The antiphospholipid syndrome (Hughes' syndrome) | ||||||||
| Last updated 01.08.2005 Written by Dr MY Karim, lecturer in immunology, St Thomas' Hospital and Dr GRV Hughes, consultant physician and rheumatologist, St Thomas' Hospital | ||||||||
| The antiphospholipid syndrome. | ||||||||
| Natl Med J India. 2003 Nov-Dec;16(6):311-6. Promotes an INR of 3-4. | ||||||||
| The expanding spectrum of renal diseases associated with antiphospholipid syndrome. | ||||||||
| Am J Kidney Dis. 2003 Jun;41(6):1205-11. The cases reported here represent a new aspect of the expanding spectrum of renal diseases encountered in association with APS. | ||||||||
| The Spectrum of the Antiphospholipid Syndrome: A Matter of Perspective | ||||||||
| © 2001. The Journal of Rheumatology Publishing Company Limited. | ||||||||
| Unusual Manifestations of the Antiphospholipid Syndrome | ||||||||
| Reported by Ronald A. AshersonI MD,FACP,MD(Hon)FRCP,FCP(SA)FACR,and Ricard Cervera2 MD,PhD | ||||||||
| Warfarin in Antiphospholipid Syndrome — Time to Explore New Horizons | ||||||||
| The Journal of Rheumatology Feb. 2005 | ||||||||
| Top of Page | ||||||||
| ||||||||
| APS - Cardiology & Pulmonology Related |
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| Antiphospholipid antibodies and hypertension. | ||||||||
| Lupus. 2004;13(10):769-72. Hypertension is a common manifestation of antiphospholipid syndrome (APS). Antiphospholipid antibodies (aPL) have been described in patients with hypertension secondary to renal artery stenosis (RAS). Twenty-six patients with RAS and 25 patients with severe essential hypertension (diastolic blood pressure > 110 mmHg or > or = 3 hypertensive drugs) were studied and compared to 61 age- and sex-matched healthy subjects. Serum samples were tested for lupus anticoagulant (LA), anticardiolipin (aCL) IgG and IgM, antiprothrombin (aPT) IgG and IgM, anti-beta2glycoprotein 1 (abeta2GP1) IgG and IgM. aPL were negative in all patients with RAS. Two patients with essential hypertension had positive aPL (8%) (LA in one patient confirmed in a second assay and abeta2GP1-IgG in the other patient confirmed one year later together with aCL IgG positivity). Among healthy subjects, one case (1.6%) was found to be positive for LA, aCL IgM, abeta2GP1 IgM, aPT IgG, aPT IgM. In conclusion, the association between RAS and aPL seems to be casual rather than an expression of an elective thrombotic localization ofAPS. The positive finding of aPL in 8% of patients with essential hypertension, a frequency higher than that of the control population, deserves further studies in larger series to better explore the relationship between aPL and hypertension. | ||||||||
| Antiphospholipid Antibodies Tied to Mitral Regurgitation in SLE | ||||||||
| Arthritis Rheum 2006;54:3918-3925. | ||||||||
| Antiphospholipid antibodies: a prognostic factor in sarcoidosis? | ||||||||
| CHEST, April, 1994. | ||||||||
| Antiphospholipid antibody syndrome as the cause of clinical rapidly progressing vasculopathy | ||||||||
| Dtsch Med Wochenschr. 2000 May 12;125(19):589-93. | ||||||||
| Antiphospholipid Syndrome and Perioperative Hemostatic Management of Cardiac Valvular Surgery | ||||||||
| Mayo Clin Proc. 2000;75:971-976 © 2000 Mayo Foundation for Medical Education and Research. Hemostatic aspects of antiphospholipid syndrome (APS) present unique challenges to clinicians and laboratory personnel alike, particularly in the perioperative period. These challenges are especially evident in patients requiring cardiac valve replacement surgery. However, the literature outlining the optimal approach in such patients is limited. We present the case of a 25-year-old woman with severe aortic regurgitation as a result of APS with particular reference to the precautions necessary during perioperative care. Particularly important are the prevention of thrombotic or hemorrhagic complications, management of associated thrombocytopenia, and laboratory methods of perioperative anticoagulation monitoring in the setting of prolonged clotting times. | ||||||||
| Antiphospholipid syndrome and vascular disease | ||||||||
| Cas Lek Cesk. 1999 Apr 26;138(9):276-9. | ||||||||
| Antiphospholipid syndrome presenting as cardiac failure | ||||||||
| Q J Med 2001; 94: 504-506 | ||||||||
| Antiphospholipid syndrome with anti-prothrombin autoantibodies in a patient with an axial-flow left ventricular assist device. | ||||||||
| J Heart Lung Transplant. 2005 Aug;24(8):1133-6. Autoantibodies to prothrombin, first described almost 50 years ago, are paradoxically associated with thrombosis. Described is an unusual case of fatal hypercoagulability in a patient with multiple arterial and venous thromboembolic complications despite intense anticoagulation while being bridged to transplantation with a left ventricular assist device. Serum analysis revealed the presence of prothrombin autoantibodies and high titers of anti-nuclear antibodies, and autopsy revealed pulmonary arteriolar vasculitis. These findings suggest an autoimmune basis for the presence of anti-prothrombin antibodies and the hypercoagulable state observed in the present case. | ||||||||
| Assessment of Cardiac Structure and Left Atrial Appendage Functions in Primary Antiphospholipid Syndrome | ||||||||
| Stroke. 2005;36:592. © 2005 American Heart Association, Inc. Conclusions— It was concluded that disease process in PAPS frequently involved cardiac valves especially mitral valve but spared LAA function. LAA function was normal, but intracardiac thrombus was present in 5 patients and 1 of them was located in LAA. MR in PAPS patients seems to impair LAA function. | ||||||||
| Association of antibodies against phospholipids with heart valve disease in systemic lupus erythematosus. | ||||||||
| Lancet. 1990 Jun 30;335(8705):1541-4. | ||||||||
| Cardiac dysfunction in SLE and antiphospholipid syndrome patients | ||||||||
| Ann Rheum Dis. Published Online First: 1 November 2006. doi:10.1136/ard.2005.044073 © 2006 by BMJ Publishing Group Ltd & European League Against Rheumatism. Conclusion: Abnormal echocardiographic findings were detected frequently in asymptomatic patients with SLE or PAPS. Although SLE patients were younger, LV systolic function was more impaired in SLE compared to PAPS patients while LV and RV diastolic function, as reflected by LVIVRT and E/A ratios, were significantly more impaired in APS patients. | ||||||||
| Cardiac involvement in the antiphospholipid syndrome. | ||||||||
| Lupus. 2005;14(9):691-6. Antiphospholipid syndrome (APS) is a systemic autoimmune disease, associated with a hypercoagulable state and fetal loss and with other clinical manifestations including cardiac involvement. Cardiac manifestations of APS are valve abnormalities (valve thickening and vegetations), occlusive arterial disease (atherosclerosis and myocardial infarction), intracardiac emboli, ventricular dysfunction, and pulmonary hypertension. Antiphospholipid antibodies (aPLs) may have a role in the accelerated atherosclerotic arterial disease observed in APS, related to their ability to induce endothelial activation. aPLs have been incriminated in the pathogenesis of heart valve lesions in APS patients. Markers of endothelial cell activation are up-regulated with prominent deposition of aPL in heart valves, suggesting aPL deposition initiates an inflammatory process that recruits complement leading to the valve lesion. Autoantibody-mediated endothelial cell activation probably plays a role in sustaining a proadhesive, proinflammatory, and procoagulant phenotype. The heterogeneity of APS clinical manifestations is likely linked to the varied effects that aPL can induce on endothelial cells and to the different functions that endothelial cells display depending on the anatomic localization. | ||||||||
| Cardiac manifestations of the antiphospholipid syndrome. | ||||||||
| Am Heart J. 1992 Nov;124(5):1331-8. The antiphospholipid syndrome has been associated with multiple cardiac abnormalities. The earliest reports were of valvular disease, including verrucous endocarditis, as well as valvular thickening and insufficiency. Subsequently, antiphospholipid antibodies were implicated in coronary artery disease manifested by premature myocardial infarction and coronary artery bypass graft occlusion. In addition, there have been rare reports of intracardiac thrombi and diffuse cardiomyopathy in association with antiphospholipid antibodies. In this review, we discuss the nature and prevalence of the cardiac manifestations of the antiphospholipid antibody syndrome as well as some of the proposed pathophysiologic mechanisms. We also provide examples from our own experience. The expanding spectrum of cardiac disease associated with antiphospholipid antibodies suggests an important role for these antibodies in certain types of cardiac pathology. | ||||||||
| Cardiac Valvulopathy in the Antiphospholipid Syndrome | ||||||||
| Source: Clinical Reviews in Allergy and Immunology, Volume 23, Number 3, December 2002, pp. 341-348(8) Abstract: The Libman and Sacks non bacterial endocarditis was reported in 1924 in patients with SLE. Its relation to the anti cardiolpin syndrome has only been described as recently as the last decade. In this paper we review the deposition of theses antibodies on the valve with complement components initiating the deformation of the valve. The valvulopathy in APS is quite common and may lead to valve replacement. In addition, a diversity of manifestations are detailed. More awareness should be drawn to this new complication of APS. | ||||||||
| Cardiovascular Pathology - Libman-Sacks endocarditis | ||||||||
| Here are flat, pale tan, spreading vegetations over the mitral valve surface and even on the chordae tendineae. This patient has systemic lupus erythematosus. Thus, these vegetations that can be on any valve or even on endocardial surfaces are consistent with Libman-Sacks endocarditis. These vegetations appear in about 4% of SLE patients and rarely cause problems because they are not large and rarely embolize. Note also the thickened, shortened, and fused chordae tendineae that represent remote rheumatic heart disease. | ||||||||
| Classification Criteria for Antiphospholipid Syndrome: The Case for Cardiac Valvular Disease | ||||||||
| © 2004. The Journal of Rheumatology Publishing Company Limited. | ||||||||
| Clinical study of anti-phospholipid antibody in patients with sarcoidosis | ||||||||
| Nihon Kyobu Shikkan Gakkai Zasshi. 1994 Jan;32(1):3-8. | ||||||||
| Heart Valve Involvement (Libman-Sacks Endocarditis) in the Antiphospholipid Syndrome | ||||||||
| Circulation. 1996;93:1579-1587. © 1996 American Heart Association, Inc. The antiphospholipid syndrome (APS) is defined by the presence of anti-phospholipid antibodies (aPLs) and venous or arterial thrombosis, recurrent pregnancy loss, or thrombocytopenia. The syndrome can be either primary or secondary to an underlying condition, most commonly systemic lupus erythematosus (SLE). Echocardiographic studies have disclosed heart valve abnormalities in about a third of patients with primary APS. SLE patients with aPLs have a higher prevalence of valvular involvement than those without these antibodies. Valvular lesions associated with aPLs occur as valve masses (nonbacterial vegetations) or thickening. These two morphological alterations can be combined and are thought to reflect the same pathological process. Both can be associated with valve dysfunction, although such association is much more common with the latter alteration. The predominant functional abnormality is regurgitation; stenosis is rare. The mitral valve is mainly affected, followed by the aortic valve. Valvular involvement usually does not cause clinical valvular heart disease. The presence of aPLs seems to further increase the risk for thromboembolic complications, mainly cerebrovascular, posed by valve lesions. Superadded bacterial endocarditis is rare but may be difficult to distinguish from pseudoinfective endocarditis. The current therapeutic guidelines are those for APS in general. Secondary antithrombotic prevention with long-term, high-intensity oral anticoagulation is advised. The efficacy of aspirin, either alone or in combination, is yet to be assessed. Corticosteroids are not beneficial and may even facilitate valve damage. Immunosuppressive agents should only be used for the treatment of an underlying condition. Current data suggest a role for aPLs in the pathogenesis of valvular lesions. aPLs may promote the formation of valve thrombi. These antibodies may also act by another mechanism, as indicated by the finding of subendothelial deposits of immunoglobulins, including anti-cardiolipin antibodies, and of colocalized complement components in deformed valves from patients with APS. | ||||||||
| Heart Valve Involvement (Libman-Sacks Endocarditis) in the Antiphospholipid Syndrome | ||||||||
| (Circulation. 1996;93:1579-1587.) © 1996 American Heart Association, Inc. Conclusion: Data provided by clinical and immunopathological studies support an association between aPLs and heart valve lesions. They also imply that aPLs play a pathogenetic role in endocardial damage. Basic and randomized, prospective, controlled clinical studies are needed to further define the role of aPLs in cardiac valve disease, elucidate its natural history, and establish optimal treatment and prevention of the disease and its potential clinical sequelae. | ||||||||
| Hypertension as the presenting feature of the antiphospholipid syndrome. | ||||||||
| Lupus. 2002;11(4):253-6. The antiphospholipid or Hughes syndrome is the association between antiphospholipid antibodies (aPL), venous and arterial thromboses and pregnancy morbidity. Antiphospholipid syndrome (APS) commonly coexists with autoimmune diseases usually systemic lupus erythematosus (SLE), when it is known as secondary APS. When present in isolation it is known as primary APS (PAPS). Although the kidney may be affected in APS, its involvement is perhaps not as well described as that of other organs. Thrombotic microangiopathy (TMA) affecting the kidney has been reported as a manifestation in both primary and secondary APS. This report describes hypertension related to underlying renal TMA as a presenting symptom of APS. | ||||||||
| Libman-Sacks endocarditis and cerebral embolization in antiphospholipid syndrome. | ||||||||
| Eur J Echocardiogr. 2008 Jan;9(1):192-3. In antiphospholipid syndrome (APS), there is a high prevalence of valvular heart disease which leads to increased risk of thrombo-embolic events, in particular, cerebrovascular events. We present a patient with cerebral infarction, previous deep-vein thrombosis, and miscarriages with positive lupus anticoagulant and anticardiolipin antibodies. Echocardiographic examination revealed mitral valve leaflet thickening and verrucous vegetations consistent with Libman-Sacks endocarditis, which is commonly associated with APS. In patients with combined Libman-Sacks endocarditis and antiphospholipid antibodies, anticoagulation therapy with warfarin is indicated due to high risk of valvular thrombus formation and subsequent embolization. | ||||||||
| Libman-sacks endocarditis and primary antiphospholipid syndrome. | ||||||||
| J Heart Valve Dis. 2005 Sep;14(5):700-2. Cardiac involvement is a not uncommon complication in patients with antiphospholipid syndrome (APS). Herein, the case is reported of cardiac failure in a female patient with Libman-Sacks endocarditis and with primary APS diagnosed eight years previously. Aggressive anticoagulation therapy and medical treatment for the cardiac failure over a 12-month period resulted in a partial regression of the severe mitral regurgitation. Close clinical and echocardiographic surveillance during the follow up of patients with APS and heart valve disease is mandatory. Optimal treatment, including adequate aggressive anticoagulation therapy and specific treatment for heart failure, may play a pivotal role in reducing the severity of valve dysfunction in these patients. | ||||||||
| LIBMAN-SACKS ENDOCARDITIS IN A PREGNANT WOMAN WITH ACUTE RESPIRATORY DISTRESS SYNDROME | ||||||||
| Obstetrics & Gynecology 1999;93:819-821 © 1999 by The American College of Obstetricians and Gynecologists. Conclusion: With rapid decompensation of acute respiratory distress syndrome in pregnancy, despite aggressive medical therapy, complicating processes must be considered, especially with antiphospholipid antibodies, which can be associated with sterile heart vegetations and subsequent fatal thromboembolism. | ||||||||
| Myocardial infarction caused by cardiac microvasculopathy in a patient with the primary antiphospholipid syndrome. | ||||||||
| Ann Intern Med. 1992 Jun 15;116(12 Pt 1):974-6. Antiphospholipid antibodies occur in various clinical states, including the primary antiphospholipid syndrome. Clinical features in these conditions appear to be caused by vasculopathy associated with the presence of these antibodies. We report the case of a patient with primary antiphospholipid syndrome who experienced cardiac necrosis secondary to myocardial microvasculopathy in the absence of vasculitis. This case demonstrates unequivocally that noninflammatory myocardial microvasculopathy occurs in the primary antiphospholipid syndrome per se without any clinical or immunologic signs of systemic lupus erythematosus or other disease process. The histopathologic findings in the skin and myocardial biopsies showed a noninflammatory vasculopathy characterized by bland thrombi and lack of infiltration of the vessel wall by inflammatory cells. Ultrastructural examination of the myocardial biopsy confirmed the vascular thrombosis and endothelial activation and showed no deposits in basement membranes. The patient survived after appropriate treatment. Evidence presented here supports the concept that the vasculopathy in the antiphospholipid syndrome is distinct from other types of vascular occlusions seen in systemic lupus erythematosus. We suggest that myocardial biopsy can be crucial in showing an underlying myocardial ischemic process despite "normal" findings on coronary angiography. Results of the biopsy hastened the decision to use potentially lifesaving plasmapheresis and anticoagulation therapy in this patient. | ||||||||
| Overt congestive heart failure with mitral and aortic regurgitation due to antiphospholipid syndrome in a patient with systemic lupus erythematosus | ||||||||
| A 51-year-old woman with overt congestive heart failure with pleural and pericardial effusion was treated with furosemide and nifedipine, leading to improvement in her condition and a decrease in effusions. An echocardiography demonstrated mitral and aortic regurgitation with mitral valve prolapse, which caused the congestive heart failure. Since leukocytopenia and lymphocytopenia with arthralgia could be observed, serological investigations were performed. She was diagnosed as having systemic lupus erythematosus (SLE) with antiphospholipid syndrome, and started on a treatment of prednisolone and aspirin. Based on the treatment, the pleural and pericardial effusion went into complete remission, indicating that the serositis related to SLE had overlapped the heart failure. Since there was no evidence of any other diseases that could be responsible for the valvular lesions, we concluded that they were due to antiphospholipid syndrome. The administration of prednisolone had no significant effect on valvular morphology or function as demonstrated by echocardiography. When patients with valvular disease are seen, a valvulopathy related to antiphospholipid syndrome should be considered as part of the differential diagnosis. | ||||||||
| Peripheral vascular disease in antiphospholipid syndrome. | ||||||||
| Thromb Res. 2004;114(5-6):509-19. Atherosclerosis has been considered an inflammatory disease based on the finding that atherosclerotic lesion contains activated T lymphocytes reacting with oxidized low-density lipoproteins (oxLDL) and heat shock proteins (HSP); it also contains autoantigens like beta2GPI, a target of antibodies occurring in an immune-mediated thrombophilia called antiphospholipid syndrome (APS). Further support to this hypothesis comes from the cross-reactivity, which occurs between antiphospholipid antibodies (aPL) and antibodies to oxLDL. Animal experiments have shown that aPL are associated with atheroma. In addition, accelerated atherosclerosis has been detected in patients with a prototype systemic autoimmune disease, such as systemic lupus erythematosus (SLE). However, the association of APS or aPL with atherosclerosis is a matter of debate due to the small numbers of patients studied, and the fact that traditional risk factors for atherosclerosis coexist. The prevalence of APS ranges from 1.7% to 6%, and that of aPL reaches to 14% among patients with peripheral vascular disease defined on the basis of clinical outcomes. On the other hand, the prevalence of asymptomatic atherosclerosis, defined in terms of plaques in ultrasonography, reaches to 15% of patients with APS compared to 9% of SLE patients and 3% of normal controls. Among SLE patients with aPL, the prevalence of plaques ranges from 6% in premenopausal women to 31% in unselected patients. Less than 10% of APS patients express premature atherosclerosis in the absence of other risk factors. Which APS patient will develop atherosclerosis is unpredictable. | ||||||||
| Prevalence of antiphospholipid antibodies in systemic sclerosis and association with primitive pulmonary arterial hypertension and endothelial injury. | ||||||||
| Clin Exp Rheumatol. 2005 Mar-Apr;23(2):199-204. CONCLUSION: We found that the prevalence of antiphospholipid antibodies in SSc patients was low. However, aCL antibodies were associated with PAH and endothelial injury. | ||||||||
| Severe valvular regurgitation and antiphospholipid antibodies in systemic lupus erythematosus: A prospective, long-term, followup study | ||||||||
| Arthritis Rheum. 2005 Jun 2;53(3):460-467 CONCLUSION: In SLE patients, the presence of high levels of IgG anticardiolipin antibodies is associated with the development of severe valvular regurgitation and with a high incidence of thromboembolic events and the need for valvular surgery. | ||||||||
| Severe, non-infectious mitral valve endocarditis after mitral valve reconstruction in a 32-year old female with primary antiphospholipid syndrome | ||||||||
| Z Kardiol. 2004 Jul;93(7):546-54. CONCLUSION: Secondary cardiac valve operations on patients with primary antiphospholipid syndrome may be successfully performed within a multidisciplinary approach. Oral anticoagulation remains the treatment of choice to prevent further thromboembolic events. | ||||||||
| Sticky Blood May Underlie Early Atherosclerosis In Men | ||||||||
| DALLAS, TX -- April 20, 1998 | ||||||||
| The Intrarenal Vascular Lesions Associated with Primary Antiphospholipid Syndrome | ||||||||
| J Am Soc Nephrol 10:507-518, 1999 © 1999 American Society of Nephrology | ||||||||
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| APS - Catastrophic Antiphospholipid Syndrome |
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| An acute multiorgan thrombotic disorder associated with antiphospholipid antibodies; two `catastrophic' cases. | ||||||||
| Ann Rheum Dis 1997;56:568-570 ( September ). Over the past decade the antiphospholipid syndrome (APS) was defined by the presence of antiphospholipid antibodies (aPL) and clinical manifestations including thrombosis, recurrent fetal loss, thrombocytopenia, chorea, livedo reticularis, heart valve lesions, and renal involvement.1 Asherson et al first drew attention to a catastrophic variant of APS (CAPS) that is characterised by multiple widespread vascular occlusions, leading to multiple organ failure and often death.2 We describe two non-systemic lupus erythematosus (SLE) patients with a strikingly similar clinical presentation of CAPS and emphasise the difficulties in differentiating CAPS from other thrombotic angiopathies. | ||||||||
| CAPS REGISTRY | ||||||||
| Last updated: 18 September 2004 | ||||||||
| Catastrophic antiphospholipid antibody syndrome | ||||||||
| Pediatric Nephrology, Volume 20, Number 7 / July, 2005, Pages 998-999. Antiphospholipid antibody syndrome (APS) is characterized by recurrent thrombosis with the presence of circulating antiphospholipid antibodies. A diagnosis of APS requires the presence of at least one clinical and one laboratory criteria (detection of aCL IgG or IgM antibodies or the presence of lupus anticoagulant on two or more consecutive occasions 6 weeks apart). A severe, rapidly progressive form characterized by clinical involvement of at least three different organ systems with histopathological evidence of small and large vessel occlusion is termed catastrophic antiphospholipid syndrome. Early recognition of APS is crucial since aggressive management can result in a favorable outcome. | ||||||||
| Catastrophic Antiphospholipid Syndrome (CAPS) | ||||||||
| Juan Javier Lichauco, M.D., Jayashree Sinha, M.D.,, and Peter Barland, M.D. February 2002 | ||||||||
| Catastrophic Antiphospholipid Syndrome and Sepsis. A Common Link? | ||||||||
| Catastrophic antiphospholipid syndrome in a 14-year-old child. | ||||||||
| Pediatr Nephrol. 2005 Apr;20(4):519-21. Epub 2005 Feb 17. Antiphospholipid syndrome (APS) is an autoimmune disease. Less than 1% of patients with APS present with life-threatening catastrophic APS (CAPS). We report here a case of CAPS in a young girl with cardiac, gastrointestinal and renal involvement. Although the management was complicated, the outcome was better than expected. We suggest that CAPS be included in the differential diagnosis of acute renal failure in children with multi-organ involvement and prolonged phospholipid-dependent coagulation time and promptly treated with immunomodulating agents and anticoagulants. | ||||||||
| Catastrophic antiphospholipid syndrome in cancer. | ||||||||
| Haematologia (Budap). 2000; 30(4):303-11. Antiphospholipid syndrome is characterized by the presence of antiphospholipid antibodies resulting in arterial and venous thromboembolism. Apart from primary cases, this syndrome is often associated with autoimmune diseases. Around 50 cases of catastrophic antiphospholipid antibody syndrome have been reported as yet. Authors describe the first case of catastrophic antiphospholipid syndrome associated with gastric cancer. Apart from presenting the clinical case, authors also discuss the possible pathomechanism of this associated disorder including the role of immunological factors, as well as antiphospholipid antibodies. | ||||||||
| Catastrophic antiphospholipid syndrome presenting as dilated cardiomyopathy with bilateral branch retinal artery thrombosis. | ||||||||
| Int J Clin Pract. 2000 Oct;54(8):550-1. Cardiac manifestation of antiphospholipid syndrome (APS) is mainly in the form of left-sided valvular insufficiency, intra-cardiac thrombi or coronary artery occlusion. Dilated cardiomyopathy is a rare but important cardiac manifestation of APS, and responds well to adequate anticoagulation and steroids. We describe a case in which APS presented with dilated cardiomyopathy and bilateral retinal artery thrombosis. | ||||||||
| Catastrophic antiphospholipid syndrome: A rare cause of disseminated microvascular thrombotic injury – a case report with pathological and molecular correlative studies | ||||||||
| Pathology International, Volume 55 Issue 3 Page 144 - March 2005. Catastrophic antiphospholipid syndrome (CAPS) is a severe and rare variant of antiphospholipid syndrome (APS) characterized by acute multiorgan failure due to small vessel thrombi in patients with positive antiphospholipid antibodies. We report a fatal case of catastrophic antiphospholipid syndrome in a young woman with a history of polymyositis and Hodgkin lymphoma. The patient was admitted to hospital because of severe foot pain following several weeks of skin ulcerations. Doppler ultrasonography showed evidence of arterial ischemia of the both lower extremities. Despite anticoagulation, immunosuppression, plasmapheresis and antibiotic therapy, she developed cutaneous gangrene, retroperitoneal hematoma, ileus, and acute respiratory and renal failure that resulted in death. Autopsy showed multifocal vascular injury and microthrombi with associated hemorrhages and infarcts in multiple organs. The patient had normal levels of functional protein C and protein S and a normal level of plasma homocysteine. Tests for common thromophilic gene mutations including prothrombin 20210, factor V Leiden 1691, and methylene tetrahydrofolate reductase 677 were negative. To our knowledge, this is the first CAPS patient with molecular studies for genetic prothrombotic mutations. Our report showed that there was no association between the development of CAPS and inherited thromophilia. | ||||||||
| Catastrophic secondary antiphospholipid syndrome with peripheral nervous system involvement: a case report. | ||||||||
| Acta Med Okayama. 2004 Apr;58(2):107-10. A 34-year-old woman was admitted to our emergency room with a high fever, abdominal pain, dyspnea and confusion. High fever and abdominal pain had first occured after a cystocele operation 5 months earlier. Later, congestive heart failure with mural thrombus formation, peripheral polyneuropathy and ischemic cerebrovascular accident were identified in clinical follow-ups, and multiple arterial and venous thromboses were seen on cranial and abdominal magnetic resonance imaging angiography. The patient's symptoms improved with anticoagulant treatment. Antiphospholipid syndrome with elevated serum anticardiolipin IgG levels was diagnosed, and ischemic peripheral polyneuropathy with axonal degeneration was determined by sural nerve biopsy. In antiphospholipid syndrome, elevated anticardiolipin antibodies appear to be the most common acquired blood protein defect causing thrombosis. Disseminated vascular thrombosis in catastrophic antiphospholipid syndrome can result in multiorgan failure with increased morbidity and mortality. It rarely occurs secondary to various infections as in the case of our patient, who suffered postoperative intraabdominal infection. It is important to note that peripheral nervous system involvement is rare in antiphospholipid syndrome. | ||||||||
| Imaging findings in the rare catastrophic variant of the primary antiphospholipid syndrome | ||||||||
| European Radiology, Volume 12, Number 3 / March, 2002, Pages 545-548. We report imaging findings in a case of the rare catastrophic variant of antiphospholipid syndrome (CAPS) characterized by widespread microvascular occlusions, which may lead to multiple organ failure. We present a case of a 66-year-old woman with bone marrow necrosis, acute acalculous cholecystitis (AAC), focal liver necrosis, subtle patchy splenic infarctions, and bilateral adrenal infarction. The demonstration of multiple microvascular organ involvement (three or more) is crucial for the diagnosis of the catastrophic variant of APS. This can be performed radiologically intra-vitam. Imaging can even reveal subclinical microinfarctions, which are often only diagnosed at autopsy. | ||||||||
| Laboratory studies on pathophysiology of the catastrophic antiphospholipid syndrome. | ||||||||
| Autoimmun Rev, December 1, 2006; 6(2): 68-71. The 'catastrophic' variant of the antiphospholipid syndrome (APS) is characterized by a diffuse thrombotic microvasculopathy. In contrast to the classical APS, single venous or arterial medium-to-large blood vessel occlusions are uncommon. The mechanisms of catastrophic APS are not clearly understood. In addition, there are no studies on pathophysiologic mechanisms of catastrophic APS. The clinical manifestations of catastrophic APS probably depend on (a) the organs affected by the thrombotic events and extent of the thrombosis and (b) manifestations of the systemic inflammatory response syndrome which are presumed to be due to excessive cytokine release from affected and necrotic tissues. The evident relationship between APS and infection may enable us to explain the development of catastrophic APS using the sepsis model. This is because catastrophic APS is characterized by multiple microvascular thrombotic events, of rapid onset, and causing multiorgan failure, a picture suggestive of septic shock, in which, there is a massive, acute inflammatory response. | ||||||||
| Long term outcome of catastrophic antiphospholipid syndrome survivors | ||||||||
| Annals of the Rheumatic Diseases 2003;62:530-533. Conclusion: Sixty six per cent of patients who survive an initial catastrophic APS event remained symptom free with anticoagulation during an average follow up of 67.2 months. Twenty six per cent of the survivors developed further APS related events and the mortality rate of these patients was about 25%. | ||||||||
| Registry Improves Understanding of Catastrophic Antiphospholipid Syndrome | ||||||||
| (2006) 2, 81-89 - Nature Clinical Practice Rheumatology. The most important clinical feature of CAPS to recognize is that, despite the high risk early on of this potentially devastating syndrome, the long-term prognosis of those patients who do survive the initial onset appears to be excellent. | ||||||||
| What is "CAPS"? | ||||||||
| Written by: Thomas L. Ortel, M.D., Ph.D., Professor of Medicine & Pathology, Duke University Medical Center | ||||||||
| Top of Page | ||||||||
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| APS - Hematology & Rheumatology Related |
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| A cross-sectional study of clinical thrombotic risk factors and preventive treatments in antiphospholipid syndrome | ||||||||
| Rheumatology 2002; 41: 924-929 © 2002 British Society for Rheumatology. Conclusion. While traditional risk factors were similar between groups, pregnancy and surgical procedures increased the risk of thrombosis. Hypertension and smoking were associated with arterial events. Possessing a combination of risk factors may increase the occurrence of arterial thrombosis but not venous thrombosis. Use of aspirin and/or hydroxychloroquine may be protective against thrombosis in asymptomatic aPL-positive individuals. | ||||||||
| Acquired Thrombophilia Antiphospholipid Antibody Syndrome Category 2 Immune Problems | ||||||||
| Acquired thrombophilia, antiphospholipid antibodies (APAs) and the antiphospholipid antibody syndrome (APS) are autoimmune conditions characterized by the presence of certain clinical features and levels of circulating APAs. | ||||||||
| Annexin and APS: the clot thickens | ||||||||
| Blood, 1 June 2006, Vol. 107, No. 11, pp. 4195-4196. | ||||||||
| Antibody Associated with Autoimmune Disorders Found to "Cluster" in Families | ||||||||
| Finding May be First Step in Preventing Premature Stroke, Heart Attack and Miscarriage | ||||||||
| Antibody Resource Page | ||||||||
| Anticardiolipin Antibodies (ACA), IgA, IgG, IgM | ||||||||
| Anticardiolipin Antibodies (ACA): Antigenic Targets and Pathophysiology | ||||||||
| Anticardiolipin Test and the Antiphospholipid (Hughes) Syndrome: 20 Years and Counting! | ||||||||
| © 2004. The Journal of Rheumatology Publishing Company Limited. | ||||||||
| Antinuclear Antibody | ||||||||
| Although access to this page is not restricted, the information found here is intended for use by medical providers. Patients should address specific medical concerns with their physicians. | ||||||||
| Antiphospholipid antibodies and venous thromboembolism | ||||||||
| Volume 86, Issue 10, pp. 3685-3691, 11/15/1995 Copyright © 1995 by The American Society of Hematology | ||||||||
| Antiphospholipid antibodies by Dr. Stephan Moll (Q/A) | ||||||||
| Q1: "My hemo just ran five new tests on me, looking for another cause of clotting. It looks as if the beta-2-glycoprotein test is an indicator of APS (= antiphospholipid antibody syndrome). My IgG and IgA are both normal, but my IgM is 34 (should be | ||||||||
| Antiphospholipid antibodies in alcoholic liver disease are influenced by histological damage but not by alcohol consumption. | ||||||||
| Lupus. 2000;9(6):451-5. | ||||||||
| Antiphospholipid Antibodies Not Predictive of Future Thrombo-Occlusive Events After Ischaemic Stroke | ||||||||
| JAMA 2004;291:576-584. | ||||||||
| Antiphospholipid antibody syndrome and polymyalgia rheumatica/giant cell arteritis | ||||||||
| Rheumatology 2000; 39: 565-567 © 2000 British Society for Rheumatology | ||||||||
| Antiphospholipid Antibody Syndrome. | ||||||||
| Curr Treat Options Cardiovasc Med. 2003 Apr;5(2):127-136. Antiphospholipid antibody syndrome (APS) is a recently defined autoimmune disorder characterized by recurrent vascular thromboses or recurrent pregnancy morbidity; these features are linked to the presence in blood of autoantibodies against negatively charged phospholipids or phospholipid-binding proteins. Thrombosis can occur in any tissue, in veins, arteries, or the microvasculature. Pregnancy morbidity in APS includes miscarriages or premature birth. Criteria that define the major clinical and laboratory features of APS were published in 1999. In patients with antiphospholipid antibodies and prior thrombosis or pregnancy morbidity, there is a high risk of recurrence that persists as long as antiphospholipid antibodies occur in blood. This risk for recurrence of thrombosis or pregnancy morbidity is greatly reduced by preventive anticoagulant therapy. Patients presenting with thrombosis in APS are initially managed in much the same way as are patients with vascular thrombosis owing to other causes. However, in patients with APS, high-intensity anticoagulation is usually needed to prevent recurrences of thrombosis. Thrombosis in APS is often multifactorial, as with non-APS thrombosis. Therefore, in all patients with APS, other reversible risk factors for thrombosis should be sought. The pregnancy outcome of women with APS who have had prior miscarriages is greatly improved by treatment during pregnancy with a combination of heparin and low-dose aspirin. | ||||||||
| Antiphospholipid Antibody Syndrome: Lupus Anticoagulants and Anticardiolipin Antibodies | ||||||||
| Antiphospholipid antibody testing: which are most useful for diagnosis? | ||||||||
| Rheum Dis Clin North Am. 2006 Aug;32(3):455-63. Laboratory diagnosis of APS relies on the demonstration of a positive test for aPL. In clinical practice, the gold standard tests are those that detect beta2GPI-dependent aCL or LA. The question on the use of anti-beta2GPI asa routine diagnostic test remains unanswered, and testing for these antibodies should be only performed in very selected cases and not as an alternative to aCL or LA testing. Clinical utility and standardization are still lacking for other aPL specificities; therefore, their application as routine diagnostic tools is not recommended. | ||||||||
| Antiphospholipid syndrome and recurrent thrombosis in children. | ||||||||
| Arthritis Rheum, November 30, 2006; 55(6): 850-855. CONCLUSION: APS in children has unique features. SLE may develop in a significant percentage of girls presenting with APS. Hereditary thrombophilia did not predict recurrent thrombosis, whereas the preventive impact of anticoagulant treatment following the first thrombotic event was noteworthy. | ||||||||
| Antiphospholipid Syndrome and Vascular Ischemic (Occlusive) Diseases: An Overview | ||||||||
| Yonsei Med J 48(6):901 - 926, 2007. DOI 10.3349/ymj.2007.48.6.901. CONCLUSION The nature of APS, as well as the evolving symptomatology of SLE, contributed to the recognition that anticoagulating approaches, rather than steroids or immunosuppressive drugs, significantly improved the outcome in a substantial number of patients with APS. We may also conclude that CNS disease in SLE is significantly associated with aPLs. Cerebral ischaemia due to vessel occlusion is considered the most important cause of CNS diseases in SLE, and aPLs play an important role in their pathogenesis. There is a strong association between aPLs and CVD, headache, cognitive dysfunction, and seizures, thus supporting the importance of occlusive vasculopathies in neuro-psychiatric lupus. Hence, testing for aPLs should be recommended not only for patients with autoimmune diseases and neuropsychiatric syndromes but also for younger patients (age < 40 years) without an underlying autoimmune disease who develop ischemic cerebral events and may have 'non-classic' MS, transverse myelitis, or atypical seizures. During brain MRI, such young individuals with multiple hyperintensity lesions without other known causes should also undergo testing for aPLs. All patients with a target INR > 3.0 and cerebral ischaemia should undergo anticoagulation therapy to prevent recurrences. All patients with thrombosis associated with aPLs should undergo long-term (life-long) warfarin therapy. Unfortunately, low-dose aspirin alone does not prevent recurrent thrombosis. Additionally, oral anticoagulation carries an increased risk of serious haemorrhage; however, this risk, if well controlled, might be lowered to acceptable levels. Steroids and immunosuppressive drugs in aPLpositive patients and thrombosis may be justified only in life-threatening situations when episodes of thrombosis occur despite adequate anticoagulation treatment. In aPL-positive patients without previous thrombosis, low-dose aspirin (75 mg/day) indefinitely as a thromboprophylactic measure is recommended. In neuropsychiatric appearances different from CVD (e.g., headache, seizures), anticoagulation therapy should be considered for patients with more severe disease and unsatisfactory responses to traditional treatments for headache or conventional anti-epileptic drugs. | ||||||||
| Antiphospholipid Syndrome from Pediatrics/Rheumatology | ||||||||
| Authored by Barry L Myones, MD, Director of Research, Pediatric Rheumatology Center, Texas Children's Hospital at Houston; Associate Professor, Departments of Pediatrics & Immunology, Pediatric Rheumatology Section, Baylor College of Medicine Adjust dose to maintain an INR in the range of 2.5-3.5. | ||||||||
| Antiphospholipid syndrome in patients with systemic lupus erythematosus treated by autologous hematopoietic stem cell transplantation. | ||||||||
| Blood. 2005 Oct 15;106(8):2700-9. Epub 2005 May 3. | ||||||||
| Antiphospholipid syndrome with only antiphosphatidylethanolamine antibodies: report of 20 cases | ||||||||
| Rev Med Interne. 2002 Apr;23(4):357-63. PURPOSE: The association of antiphosphatidylethanolamine antibodies (aPE) as the only antiphospholipid antibody with antiphospholipid syndrome (APS) is discussed. The aPE was described as the sole antibody in many cases suggesting APS. aPE was not included in the Sapporo criteria for the classification of APS. METHODS: We investigated the clinical features of 20 patients with aPE only; 17 patients had symptoms potentially related to APS (group 1) and three had other manifestations (group 2). RESULTS: There were 15 women and five men, mean age was 35 +/- 12 years at the beginning. In group 1 (n = 17), ten patients presented arterial thrombosis, nine venous thrombosis (five had both), and six microvascular thrombosis (livedo reticularis, lacunar pathology). The aPE positivity was persistent in 13 patients. A subgroup of four patients (three women) presented arteriosclerosis with peripheral arteriopathy which started before 45 years of age. They had another atherosclerosis risk factor associated with aPE persistence. In group 2 (n = 3), there was no thrombotic event, one demyelinating pathology, one microvascular pathology, and one arterial dysplasia. The aPE positivity was never confirmed. Finally, 13 patients presented an APS with aPE only, confirmed at least 8 weeks later. CONCLUSIONS: Our study points out that testing for aPE would be of interest for patients when symptoms were potentially related to APS, particularly when other antiphospholipid antibodies were negative. This description questions the enlargement of the APS biological criteria defined in Sapporo. The role of aPE in atherosclerosis is considered. | ||||||||
| Antiphospholipid syndrome without antiphospholipid antibodies at the time of the thrombotic event: transient 'seronegative' antiphospholipid syndrome? | ||||||||
| Clin Exp Rheumatol. 1997 Sep-Oct;15(5):541-4. The antiphospholipid syndrome (APS) is characterized by the presence of venous and arterial thrombosis, recurrent fetal losses and thrombocytopenia, associated with the presence of antiphospholipid antibodies (aPL). This syndrome may be "primary" or may be associated with other diseases, mainly systemic lupus erythematosus (SLE). However, some patients present the clinical picture of this syndrome but without evidence of aPL in their serum. The term "seronegative" APS has been proposed to categorize these patients. Here with we present two patients with seronegativity for aPL at the time of a thrombotic event, but in whom these antibodies were detected 2 and 7 months later. | ||||||||
| Antiphospholipid Syndrome: A Coagulation Disorder in Women | ||||||||
| Posted 01/24/1997 Antiphospholipid (aPL) syndrome, or APS,--a cluster of conditions that includes arterial or venous thromboses and thrombocytopenia, as well as recurrent fetal loss associated with elevation of aPL antibody--has been reported to occur 2-5 times more frequently in women than men. Strong familial associations lead to the suspicion that aPL positivity, estimated to be present in 2% of the population, is a heritable trait in some cases. Currently, 2 major categories of the illness are recognized--primary and secondary. Secondary APS may be associated with autoimmune disease, malignancy, infectious disease, or drug-induced states. Two assays, one for lupus anticoagulant antibodies and the other for anticardiolipin (aCL) antibodies, are recognized to be the gold standards for serologic diagnosis of the disease. Despite extensive attempts at international standardization of aCL test results, no consensus exists for a value beyond which the test is considered positive. Interestingly, a "dose-effect" relationship for aCL antibody titers has been noted--higher titers of the antibody correlate with increased numbers of thrombotic events. An experimental assay for antibody against beta 2-glycoprotein 1 (beta-2-GP1), a phospholipid-binding protein, may become the most important assay for aPL. Skin findings in APS include livedo reticularis, ulceration, gangrene, or purpura, and, when present, may be the key to diagnosis of this sometimes insidious syndrome. Anticoagulation, usually with warfarin, is the mainstay of therapy, although steroids, immunosuppressive agents, hydroxychloroquine sulfate, and plasmapheresis may all be beneficial adjunctive therapy. | ||||||||
| Antiphospholipid syndrome: Clinical and immunologic manifestations and patterns of disease expression in a cohort of 1,000 patients | ||||||||
| APS may affect any organ of the body and display a broad spectrum of manifestations. An association with SLE, the patient's sex, and the patient's age at disease onset can modify the disease expression and define specific subsets of APS. | ||||||||
| Antiphospholipid thrombosis: clinical course after the first thrombotic event in 70 patients. | ||||||||
| Ann Intern Med. 1992 Aug 15;117(4):303-8. CONCLUSIONS: Recurrent thrombosis is a potentially serious problem for patients with lupus anticoagulant or anticardiolipin antibodies or both. The site of the first event (arterial or venous) tended to predict the site of subsequent events. Intermediate- to high-intensity warfarin therapy may confer better antithrombotic protection than low- to intermediate-intensity warfarin therapy or aspirin therapy. Further studies are needed to define more precisely the rethrombosis rate and optimal type, intensity, and duration of antithrombotic therapy. | ||||||||
| Antiphospholipid, anti-beta 2-glycoprotein-I and anti-oxidized-low- density-lipoprotein antibodies in antiphospholipid syndrome | ||||||||
| QJM, Vol 91, Issue 9 619-626, Copyright © 1998 by Oxford University Press | ||||||||
| Archives of Rheumatology | ||||||||
| The On-line Archives of Rheumatology publishes original papers dealing with the clinical manifestations, laboratory investigations and the treatment of rheumatic diseases. | ||||||||
| Chronic fatigue syndrome and/or Fibromyalgia as a variation of Antiphospholipid antibody syndrome: an explanatory model and approach to laboratory diagnosis | ||||||||
| D. Berg, L. H. Berg, J. Couvaras and H. Harrison (Received 22 June 1999; accepted 2 July 1999) | ||||||||
| Comparison of the primary and secondary antiphospholipid syndrome: a European Multicenter Study of 114 patients. | ||||||||
| Am J Med. 1994 Jan;96(1):3-9. | ||||||||
| Factor XIII in Primary Antiphospholipid Syndrome | ||||||||
| PAUL R.J. AMES, LUIGI IANNACCONE, JOSE DELGADO ALVES, ANNAMARIA MARGARITA, LUIS R. LOPEZ, and VINCENZO BRANCACCIO J Rheumatol 2005;32:1058-62 Conclusion. Enhanced FXIII activity may contribute to atherothrombosis in primary APS via increased fibrin/fibrinogen cross-linking. This pathway is not exclusive to primary APS, being present also in thrombotic controls, but the presence of IgG aPL may favor a higher degree of FXIIIa activation in the primary APS group. | ||||||||
| Family testing for genetic abnormalities | ||||||||
| Last Updated: 2/15/2004 | ||||||||
| Further evidence of false negative screening for lupus anticoagulants | ||||||||
| Thrombosis Research. Volume 121, Issue 4, 2008, Pages 477-484. Conclusions: LA activity can be demonstrated by assessment of screen and confirm data irrespective of screening test elevation above a reference range. Other workers have demonstrated this phenomenon in APTT using different study designs and it may be that standard interpretation criteria warrant re-assessment. | ||||||||
| General Management of the Patient with a Positive Antiphospholipid Antibody Test: What Evidence Is Available For You and Your Physician To Consider? | ||||||||
| Written by: Gale A McCarty, MD, FACP, FACR You are an individual and your particular case may involve some features that separate you from the study patients. What is most likely to have the right balance of "helpful vs. harmful" effects on you is the major concern of your physician to prevent the effects of aPL antibodies in contributing to blood clots and cell or organ damage in you. Let's look at the major approaches used to manage your positive aPL test. | ||||||||
| General Management of Thromboses (Blood Clot) II—What Evidence is Available For You and Your Physician To Consider? | ||||||||
| Written by: Gale McCarty, MD, FACP, FACR. As always, the physician treating the individual APS patient has to balance the wisdom of these guidelines (which are general guidelines and not mandates applicable to ALL patients) with the needs of his/her patient to find the best answer. | ||||||||
| Genetic study on Familial Antiphospholipid Syndrome. | ||||||||
| Antiphospholipid syndrome (APS) is familial in 10% of cases. | ||||||||
| HYDROXYCHLOROQUINE - EVERYTHING OLD IS NEW AGAIN! | ||||||||
| By: Gale McCarty, MD, FACR, FACP. Hydroxychloroquine (HCQ, or its trade name-Plaquenil) has a long and honored history of use in systemic lupus erythematosus (SLE) as a general medication to decrease activity of the immune system and decrease symptoms. For years it has been approved for use by the FDA for lupus and rheumatoid arthritis, and has been used most frequently for skin and joint manifestations. It is considered a mainstay of therapy for any patient with SLE by many lupus experts and rheumatologists. It has many mechanisms of action, some related to decrease in the activity of the immune system, and some related to effects on blood clotting mechanisms. HCQ belongs to the class of drugs call anti-malarials, which includes Chloroquine and Atabrine. (This does not mean that anyone thinks that SLE or APS is caused by the agent that causes malaria-like most discoveries in medicine, it was the chance observation that patients with some autoimmune diseases who got anti-malarial drugs to prevent malaria when traveling to likely areas of infection noted their symptoms improved on HCQ). One of the most complete and excellent reviews of all the literature on the anti-malarials to which all patients and their physicians are directed is Dr. Dan Wallace's Chapter 59 in the Wallace-Hahn Dubois' Lupus Erythematosus textbook. Another excellent review on APS therapy in general has been published by Dr. Robert Roubey. | ||||||||
| Hydroxychloroquine Reverses Thrombogenic Properties of Antiphospholipid Antibodies in Mice | ||||||||
| Circulation. 1997;96:4380-4384. Conclusions Hydroxychloroquine significantly diminished both thrombus size and total time of thrombus formation in mice previously injected with IgG-APS. | ||||||||
| Hypercoagulable State in Patients With Antiphospholipid Syndrome Is Related to High Induced Tissue Factor Expression on Monocytes and to Low Free Protein S | ||||||||
| (Arteriosclerosis, Thrombosis, and Vascular Biology. 1996;16:1319-1326.) © 1996 American Heart Association, Inc. | ||||||||
| Intravenous immunoglobulin therapy of antiphospholipid syndrome | ||||||||
| Rheumatology 2000; 39: 421-426 © 2000 British Society for Rheumatology | ||||||||
| Laboratory diagnosis and management challenges in the antiphospholipid syndrome. | ||||||||
| Lupus. 2006;15(3):172-8. The antiphospholipid syndrome (APS) is characterized by recurrent arterial and/or venous thrombosis and pregnancy morbidity manifested by early or late losses. Laboratory diagnosis ofAPS relies on the demonstration of a positive test for antiphospholipid antibodies (aPL). In clinical practice, the gold standard tests are those that detect anticardiolipin antibodies (aCL) and/or the lupus anticoagulant (LA). Although other specificities for aPL have been described their clinical utility and standardization has still to be established. Persistence of aPL positive tests must be demonstrated, and other causes and underlying factors considered. Although it is universally recognized that the routine screening tests (aCL and/or LA) might miss some cases, careful differential diagnosis and repeat testing are mandatory before the diagnosis of 'seronegative APS' can be made. Correct identification of patients with APS is important, because prophylactic anticoagulant therapy can prevent thrombosis from recurring, and treatment of affected women during pregnancy can improve fetal and maternal outcome. | ||||||||
| Livedo Reticularis in Antiphospholipid Antibody Syndrome | ||||||||
| Posted on: Tuesday, 14 February 2006, 03:03 CST | ||||||||
| LJP 1082: a toleragen for Hughes syndrome | ||||||||
| Lupus, Vol. 13, No. 5, 335-338 (2004). DOI: 10.1191/0961203304lu1022oa. © 2004 SAGE Publications | ||||||||
| Lupus anticoagulant and INR by Dr. Stephan Moll (Q/A) | ||||||||
| Example: Q1: "Could you please explain more about the effect of lupus anticoagulants on the INR tests? My hematologist has not heard of the difficulties of the two interacting with each other. Are there any references you could provide so I can give them to my doc? I have not been able to maintain a consistent INR level; it is either at 1.8 or 3.8, very rarely does it come in between 2-3." | ||||||||
| Lupus Anticoagulant Assays: Questions Answered and to Be Answered | ||||||||
| Arch Pathol Lab Med—Vol 131, June 2007. Conclusions: Although there are published criteria for confirming the presence of a lupus anticoagulant, there is no consensus on assay methods for lupus anticoagulant. | ||||||||
| Lupus Anticoagulant: Testing While on Anticoagulant Therapy: Can It Be Done? | ||||||||
| Written by: Thomas L Ortel, MD, PhD. In conclusion, it is optimal to test for a lupus anticoagulant when the patient is on no anticoagulant therapy. All of the test results can be interpreted more easily in that setting. Sometimes this can be difficult to arrange, however, and testing needs to be performed while the patient is still taking anticoagulants. In this situation, the doctor needs to work carefully with the laboratory, to understand how the tests are being performed and to make sure that the results are interpreted correctly. | ||||||||
| Lupus anticoagulants | ||||||||
| Update Date: 1/26/2005 Updated by: Rita Nanda, MD., Department of Hematology/Oncology, University of Chicago Medical Center, Chicago, IL. Review provided by VeriMed Healthcare Network. | ||||||||
| Lupus anticoagulants are stronger risk factors for thrombosis than anticardiolipin antibodies in the antiphospholipid syndrome: a systematic review of the literature | ||||||||
| Blood, 1 March 2003, Vol. 101, No. 5, pp. 1827-1832 | ||||||||
| Mechanisms of Disease: antiphospholipid antibodies—from clinical association to pathologic mechanism | ||||||||
| Nature Clinical Practice Rheumatology. Published online: 19 February 2008. doi:10.1038/ncprheum0740 Received 6 September 2007. Accepted 4 December 2007. Conclusion and future directions: APS is still seen as a rather obscure disease despite extensive research; this view is mainly the result of the unreliability of the current assays for detecting the presence of antiphospholipid antibodies. The consequences are a poor correlation between serological markers and clinical manifestations, and a lack of clarity about the pathogenetic mechanism causing the syndrome. As mentioned above, we found that the presence of LA-inducing anti-beta2 GPI antibodies (with an affinity for domain I) correlates almost uniformly with the occurrence of thrombosis.21, 23 Our next aim is to investigate whether the presence of anti-domain I antibodies correlates with recurrent fetal loss. Research focused specifically on anti-domain I antibodies will hopefully provide information about the mechanism that causes APS. We have found that these antibodies cause increased (factor V Leiden-independent) APC resistance, increased resistance against the anticoagulant properties of annexin A5 and increased levels of activated von Willebrand factor (Figure 3).39, 42, 46 It is anticipated that this population is also involved in disease-causing mechanisms other than thrombosis in APS. It seems strange that new roles for a protein with no previous clear function come to light in the presence of an autoantibody. Perhaps native beta2 GPI already possesses these functions, but requires activation (such as a conformational change) before it can perform them. Native beta2 GPI could potentially already exhibit these actions at sites of cellular damage.47 Under apoptotic conditions, the antigen would be concentrated on the anionic phospholipids of the exposed cellular membranes and inhibit the unwanted consequences of the exposure of negatively-charged surfaces to components of the circulating bloodstream. In the presence of anti-beta2 GPI antibodies, the active conformation of the beta2 GPI protein is stabilized and its function is no longer limited to sites of damage. The consequence would be an imbalance in the hemostatic system, resulting in thrombosis at any vascular site in the body. In the future, more insight is needed into whether there are more antibody subpopulations that can cause the (specific) clinical manifestations that are observed in APS. Future investigations into the pathology should be performed with antibody populations with precisely defined epitopes. | ||||||||
| Monitoring Warfarin Therapy in Patients with Lupus Anticoagulants | ||||||||
| Recommended therapeutic international normalized ratios (INRs) for oral anticoagulation in patients with lupus anticoagulants who sustain a thromboembolic event are controversial. Patients with lupus anticoagulants often have a prolonged prothrombin time, which may complicate management of anticoagulant therapy. | ||||||||
| Multiple antiphospholipid tests do not increase the diagnostic yield in antiphospholipid syndrome | ||||||||
| The British Journal of Rheumatology, Vol 37, 1229-1232, Copyright © 1998 by British Society for Rheumatology | ||||||||
| New approaches to prevention of thrombosis in the antiphospholipid syndrome: hopes, trials, and tribulations. | ||||||||
| Arthritis Rheum. 2003 Nov;48(11):3004-8. | ||||||||
| Ovarian Vein Thrombosis May Occur in Antiphospholipid Syndrome, May Be Underdiagnosed | ||||||||
| Arthritis Rheum 2004 Jan;50:1:183-6. "Ovarian vein thrombosis in the antiphospholipid syndrome" | ||||||||
| Pathogenesis of the antiphospholipid syndrome: An additional example of the mosaic of autoimmunity | ||||||||
| Journal of Autoimmunity. Volume 30, Issues 1-2, February-March 2008, Pages 99-103. Autoimmunity: From the Mosaic to the Kaleidoscope. The antiphospholipid syndrome (APS) is a systemic autoimmune disease characterized by an adaptive immune response against self-PL-binding proteins ending in the production of specific autoantibodies. Antiphospholipid antibodies (aPL; and in particular anti-β2 glycoprotein I antibodies) are formal diagnostic markers and pathogenic antibodies. Although APS may be considered as an autoantibody-mediated disease, there is now evidence that aPL are necessary but not sufficient to trigger some of the clinical manifestations of the syndrome. For example, additional factors, such as mediators of the innate immunity are now recognized to play a key role as second hits able to induce the thrombotic events in the presence of the autoantibodies. The APS scenario is also supplemented by the influence of genetically determined factors. Finally, environmental agents – in particular infectious ones – were reported to act as triggers for the production of autoantibodies cross-reacting with PL-binding proteins as well as inflammatory stimuli that potentiate the aPL thrombogenic effect. Altogether these findings do support the concept of a mosaic of factors that participate to the pathogenesis of the syndrome at different levels. | ||||||||
| Patient information: Blood clotting problems due to antibodies (antiphospholipid antibody syndrome) | ||||||||
| Peter H Schur, MD Harvard Medical School UpToDate performs a continuous review of over 330 journals and other resources. Updates are added as important new information is published. The literature review for version 13.2 is current through April 2005; this topic was last changed on July 6, 2004. The next version of UpToDate (13.3) will be released in October 2005. | ||||||||
| Patients with antiphospholipid antibodies and venous thrombosis should receive long term anticoagulant treatment | ||||||||
| Annals of the Rheumatic Diseases, 1993, Vol 52, 689-692 | ||||||||
| Platelet-Endothelial Interactions: Sepsis, HIT, and Antiphospholipid Syndrome | ||||||||
| Hematology 2003 © 2003 The American Society of Hematology | ||||||||
| Possible mechanisms of action of the antiphospholipid binding antibodies. | ||||||||
| Clin Exp Rheumatol. 1989 Sep-Oct;7 Suppl 3:S85-9. If antibodies directed against the phospholipid itself play any part in the pathogenesis of the thrombotic events, the very ubiquitous nature of the antigen dictates that no single pathogenetic mechanism can be implicated. The majority of in vivo studies recently performed have failed to show that antiphospholipid (aPL) antibodies bind cultured endothelial cells, or that they affect prostacyclin production by these cells. Previously postulated actions showing defective fibrinolysis and impairment of coagulation inhibitors (protein C, thrombomodulin and antithrombin III) have yet to be substantiated. Studies have thus far failed to demonstrate any significant binding of aPL antibodies to intact platelets, although binding to disrupted platelets has been documented. | ||||||||
| Predicting Thrombosis Risk in Individuals with Antiphospholipid Antibodies | ||||||||
| Written by: Thomas L Ortel, MD, PhD "...several studies have shown that the presence of a lupus anticoagulant in the blood is associated with a higher risk for a clot than the presence of an anticardiolipin antibody." | ||||||||
| Primary antiphospholipid syndrome and panniculitis. | ||||||||
| On-line Arch Rheumatol 1998; 2: 4 published on June 7, 1998 | ||||||||
| RESULTS OF A RANDOMIZED, PLACEBO CONTROLLED, DOUBLE BLIND PHASE 1/2 CLINICAL TRIAL (RCT) TO ASSESS THE SAFETY AND TOLERABILITY OF LJP 1082 IN PATIENTS WITH ANTIPHOSPHOLIPID SYNDROME | ||||||||
| Arash Horizon, Michael H. Weisman, Daniel J. Wallace, Joan T. Merrill, Matthew D. Linnik, Keith A. Cockerill, Richard Furie. Conclusion: LJP 1082 appeared to be well tolerated in patients with antibodies to beta-2-GPI following a single intravenous dose up to 200 mg. Although the single-dose trial design was not intended to measure B cell tolerance, the observed binding of LJP 1082 to target antibodies was consistent with its intended pharmacological activity. The results of this trial support the further development of a B cell toleragen to treat Antiphospholipid Syndrome. | ||||||||
| SERONEGATIVE ANTIPHOSPHOLIPID ANTIBODY SYNDROME (SNAPS)…AND SNAPPING TO IT!! | ||||||||
| By: Gale McCarty, MD, FACR, FACP. "You don't have the syndrome because your tests are low level or negative…" or "You have livedo, a heart valve problem, and thrombocytopenia, but these aren't listed as criteria for diagnosis" are comments made frequently by healthcare providers from many specialties to patients with clinical features suggesting the Antiphospholipid Antibody Syndrome (APS). | ||||||||
| Significance of Persistent Antiphospholipid Antibodies in the Elderly | ||||||||
| First Release July 1 2006; J Rheumatol 2006;33:1559–62. Conclusion. Interpretation of a positive determination of APL is difficult in the elderly; persistent LAC may be the most valuable biological marker of APS in the elderly. | ||||||||
| Significance of Persistent Antiphospholipid Antibodies in the Elderly | ||||||||
| Conclusion. Interpretation of a positive determination of APL is difficult in the elderly; persistent LAC may be the most valuable biological marker of APS in the elderly. (First Release July 1 2006; J Rheumatol 2006;33:1559–62) | ||||||||
| Small vessel thrombosis without major thrombotic events in systemic lupus erythematosus patients with antiphospholipid syndrome. | ||||||||
| Wien Klin Wochenschr. 2000 Aug 25;112(15-16):707-10. Antiphospholipid syndrome has been defined by the presence of antiphospholipid antibodies or lupus anticoagulant in association with certain clinical events, including recurrent arterial or venous thromboses and recurrent fetal loss. It comprises two separate clinical entities: simple, characterized by large vessel occlusions, and catastrophic, with multiple occlusive events predominantly affecting small vessels. Three patients with systemic lupus erythematosus and permanently increased IgG anticardiolipin antibody levels are being described. Only postmortem histopathological examination revealed microangiopathic thrombotic changes in different organs, which were clinically silent in early stages of the disease and misinterpreted later in its course because of a peculiar clinical picture. All patients presented features of catastrophic antiphospholipid syndrome in the final stage of the disease. | ||||||||
| Sorting Out the "Alphabet Soup" of Antiphospholipid Antibody Syndrome: How Do APS, CAPS, and MAPS Relate? | ||||||||
| Written by: Gale A. McCarty MD FACP FACR, Chief of Rheumatology and Immunotherapy/Center for Lupus and APS, Maine Coastal Memorial Hospital | ||||||||
| Testing for and Clinical Significance of Anticardiolipin Antibodies | ||||||||
| Clinical and Diagnostic Laboratory Immunology, November 1999, p. 775-782, Vol. 6, No. 6 1071-412X/99/$04.00+0. The aCL assay is only one of the methods used to detect aPL, and the test should be administered with the LA and anti-B2GPI assays. The aCL assay is reasonably sensitive but not at all specific, with additional significant variation among laboratories and among commercial kits; therefore, clinicians should treat the clinical state and not an incidentally found antibody. Although there is an association between antibody titer and risk of thrombosis, this is not a ground for ignoring or not reporting weakly positive results. False-positive results that are difficult to interpret are particularly likely to occur when there are other causes of thrombosis such as atherosclerosis in the elderly; therefore, screening widely should not be encouraged. The predictive value of testing with the aCL ELISA for APS can be improved to an extent by concurrent LA testing, but often the appropriate duration and intensity of treatment after a first manifestation cannot be determined by these two tests. The anti-B2GPI ELISA offers an improved predictive value, but there is a need for an optimization or evaluation procedure to manipulate and assess the effects of the different variables on test performance. We suggest that laboratories interested in aPL evaluate the assay, both in an in-house form using an optimal buffer and a large quantity of B2GPI and in a commercial form when thoroughly tested and externally validated kits become available. | ||||||||
| Testing for and Clinical Significance of Anticardiolipin Antibodies | ||||||||
| Clinical and Diagnostic Laboratory Immunology, November 1999, p. 775-782, Vol. 6, No. 6 | ||||||||
| The antiphospholipid antibody syndrome in the emergency department setting--livedo reticularis and recurrent venous thrombosis. | ||||||||
| Ann Emerg Med. 1992 Feb;21(2):207-11. We present the case of a 26-year-old man with an exacerbation of apparent chronic asthma with chronic peripheral vascular disease due to recurrent venous thrombosis. Localized livedo reticularis, new cutaneous infarctions, severe venous insufficiency, thrombocytopenia, renal failure, and cerebral supratentorial dysfunction were noted. During hospital admission, antibodies to phospholipids in high titer were present by three different testing methods. Renal biopsy demonstrated significant renal vasculature abnormalities characteristic of hemolytic endovasculopathy, and magnetic resonance imaging showed multiple cerebral infarctions. This case exemplifies the spectrum of presentations and management of the primary antiphospholipid antibody syndrome. The clue to its presence in this patient was the livedo reticularis rash, a cutaneous marker for this syndrome that was evident in the emergency department. | ||||||||
| The Management of Thrombosis in the Antiphospholipid-Antibody Syndrome | ||||||||
| N. Engl. J. Med. 332:993-997 April 1995. Conclusions The risk of recurrent thrombosis in patients with the antiphospholipid-antibody syndrome is high. Long-term anticoagulation therapy in which the international normalized ratio is maintained at or above 3 is advisable in these patients. | ||||||||
| The Presence of Multiple Prothrombotic Risk Factors Is Associated with a Higher Risk of Thrombosis in Individuals with Anticardiolipin Antibodies | ||||||||
| J Rheumatol 2003;30:2385-91. Conclusion. In individuals with positive aCL-IgG, we observed an association between the number of prothrombotic risk factors and history of thrombotic events. | ||||||||
| Thrombocytopenia in the Antiphospholipid Syndrome | ||||||||
| SJS Aug 2003 | ||||||||
| Thrombosis and the Antiphospholipid Syndrome | ||||||||
| Hematology 2005 © 2005 The American Society of Hematology. Summary: Even with the most complete datasets, it is still important for the physician to develop a therapeutic plan appropriate for the individual patient, based on clinical presentation, co-morbid conditions, and other variables. With uncommon disorders and limited datasets, such as with the antiphospholipid syndrome, decision-making becomes even more difficult. Table 3 presents a strategy that the author uses when evaluating and developing a treatment plan for a patient with antiphospholipid syndrome and thrombosis, based on the available studies summarized in this article. Critical areas for future research include identifying which patients with antiphospholipid antibodies are at highest risk for thrombotic complications, developing new antithrombotic agents that are effective and safe, and investigating novel approaches to eliminate the autoantibody and, hopefully, the increased prothrombotic state. | ||||||||
| Thrombotic microangiopathic haemolytic anaemia and antiphospholipid antibodies | ||||||||
| Annals of the Rheumatic Diseases 2004;63:730-736 © 2004 by BMJ Publishing Group Ltd & European League Against Rheumatism | ||||||||
| Use of Intravenous Immune Globulin in Antiphospholipid Syndrome | ||||||||
| Written by: Gretchen F Kunze, Pharm.D | ||||||||
| Use of Rituximab in the Antiphospholipid Syndrome | ||||||||
| Written by: Robert A. S. Roubey, MD University of North Carolina at Chapel Hill | ||||||||
| Validation of the Sapporo criteria for antiphospholipid syndrome. | ||||||||
| Arthritis Rheum. 2000 Feb;43(2):440-3. Some patients with false-negative results were true seronegative cases. CONCLUSION: The Sapporo criteria for APS compare favorably with the American College of Rheumatology criteria for SLE and are usable for clinical studies. | ||||||||
| Warfarin in Antiphospholipid Syndrome — Time to Explore New Horizons | ||||||||
| © 2005. The Journal of Rheumatology Publishing Company Limited. | ||||||||
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| APS - Lupus Related |
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| Antiphospholipid antibodies and cerebral lupus. | ||||||||
| Ann N Y Acad Sci. 1997; 823:270-8 (ISSN: 0077-8923) Antiphospholipid antibodies probably play a minor role in cerebral lupus. They are associated primarily with stroke and transient ischemic attacks, which occur only in a minority of patients with central nervous system complications of SLE. However, ready demonstration of functional effects of antiphospholipid antibodies in the coagulation system as well as persuasive evidence that they can induce thrombosis and pregnancy loss in experimental mice lend credence to the belief that many autoantibodies play a direct role in disease pathogenesis. Hence the role of the many autoantibodies associated with CNS lupus is a legitimate field of inquiry. This review outlines the history of antiphospholipid antibodies, discusses the controversy concerning antiphospholipid antibody specificity, summarizes recent experimental data on their functional effects in the coagulation system, and describes animal models of the antiphospholipid syndrome in which antibodies with cardiolipin-binding activity have been shown to play a direct role in thrombosis and pregnancy loss. | ||||||||
| ANTIPHOSPHOLIPID ANTIBODIES AND SYSTEMIC LUPUS ERYTHEMATOSUS | ||||||||
| Michelle Petri, M.D., M.P.H. Associate Professor of Medicine The Johns Hopkins University School of Medicine Baltimore, MD | ||||||||
| Antiphospholipid Antibodies: Anticardiolipin Antibodies and the Lupus Anticoagulant in Systemic Lupus Erythematosus | ||||||||
| Michelle Petri, M.D., M.P.H. Associate Professor of Medicine, The Johns Hopkins University School of Medicine Baltimore, MD | ||||||||
| Antiphospholipid syndrome in systemic lupus erythematosus: is the whole greater than the sum of its parts? | ||||||||
| Rheum Dis Clin North Am. 2005 May;31(2):255-72 | ||||||||
| Antiphospholipid Syndrome With Catastrophic Bleeding and Recurrent Ischemic Strokes as Initial Presentation of Systemic Lupus Erythematosus. | ||||||||
| J Pediatr Hematol Oncol. 2005 Jul;27(7):403-407 | ||||||||
| Antiphospholipids & SLE: A Clinical Overview | ||||||||
| Dr. Graham R.V. Hughes, MD FRCP | ||||||||
| Arterial disease in lupus and secondary antiphospholipid syndrome: association with anti-beta2-glycoprotein I antibodies but not with antibodies against oxidized low-density lipoprotein | ||||||||
| The British Journal of Rheumatology, Vol 37, 883-888, Copyright © 1998 by British Society for Rheumatology | ||||||||
| Association between antiphospholipid antibodies and epilepsy in patients with systemic lupus erythematosus. | ||||||||
| Arthritis Rheum. 1994 Apr;37(4):568-71. | ||||||||
| Association between microscopic brain damage as indicated by magnetization transfer imaging and anticardiolipin antibodies in neuropsychiatric lupus | ||||||||
| Arthritis Research & Therapy 2006, 8:R38 doi:10.1186/ar1892. The pathogenetic role of anticardiolipin antibodies (aCLs) in patients with neuropsychiatric systemic lupus erythematosus (NPSLE) without cerebral infarcts remains elusive. Magnetization transfer imaging (MTI) has proved to be a sensitive tool for detecting diffuse microscopic brain damage in NPSLE patients. In this study we examined the correlation between grey and white matter magnetization transfer ratio (MTR) parameters and the presence of IgM and IgG aCLs and lupus anticoagulant in 18 patients with systemic lupus erythematosus and a history of NPSLE but without cerebral infarcts on conventional magnetic resonance imaging. Lower grey matter mean MTR (P < 0.05), white matter mean MTR (P < 0.05), white matter peak location (P < 0.05) and grey matter peak location (trend toward statistical significance) were observed in IgM aCL-positive patients than in IgM aCL-negative patients. No significant differences were found in MTR histogram parameters with respect to IgG aCL and lupus anticoagulant status, nor with respect to anti-dsDNA or anti-ENA (extractable nuclear antigen) status. This is the first report of an association between the presence of aCLs and cerebral damage in grey and white matter in NPSLE. Our findings suggest that aCLs are associated with diffuse brain involvement in NPSLE patients. | ||||||||
| Association of mannose-binding lectin gene polymorphisms with antiphospholipid syndrome, cardiovascular disease and chronic damage in patients with systemic lupus erythematosus | ||||||||
| Rheumatology Advance Access originally published online on June 26, 2006 Rheumatology 2007 46(1):76-80; doi:10.1093/rheumatology/kel199. Conclusion: Although the prevalence of cardiovascular disease in our SLE patients carrying MBL-deficient genotypes was 3.3 times higher than in patients with non-deficient genotypes, only APS was independently associated with cardiovascular events. This suggests that the higher frequency of thrombotic events in SLE patients carrying MBL-deficient genotypes might be related to coexisting APS. | ||||||||
| Autoantibodies to human prothrombin and clinical manifestations in 207 patients with systemic lupus erythematosus. | ||||||||
| J Rheumatol. 1998 Jun;25(6):1104-8. | ||||||||
| Bone infarcts in a woman with systemic lupus erythematosus and antiphospholipid antibody syndrome | ||||||||
| CMAJ • February 14, 2006; 174 (4). doi:10.1503/cmaj.050801. © 2006 CMA Media Inc. or its licensors | ||||||||
| Clinical and laboratory diagnosis of heart disease in systemic lupus erythematosus | ||||||||
| Arq Bras Cardiol. 1997 Feb;68(2):79-83. CONCLUSION: Myocardial disease was the most frequent heart involvement in active SLE, and we did not found any association between SLE heart disease and positive anticardiolipin antibodies. | ||||||||
| High Impact of Antiphospholipid Syndrome on Irreversible Organ Damage and Survival of Patients With Systemic Lupus Erythematosus | ||||||||
| Arch Intern Med. 2004;164:77-82. CONCLUSIONS: Antiphospholipid syndrome with thrombotic manifestations is a major predictor of irreversible organ damage and death in patients with SLE. | ||||||||
| I Have the Lupus Anticoagulant, But I Don't Have Lupus? | ||||||||
| By: Thomas L. Ortel, MD, PhD "...although the 'lupus' anticoagulant was first described in several patients with lupus, most patients with lupus anticoagulants actually don't have any of the other clinical manifestations of lupus" | ||||||||
| Long-Term Follow-Up in 128 Patients With Primary Antiphospholipid Syndrome: Do They Develop Lupus? | ||||||||
| Medicine. 84(4):225-230, July 2005. Gomez-Puerta, Jose A. MD; Martin, Helena MD; Amigo, Mary-Carmen MD; Aguirre, Maria A. MD; Camps, Maria T. MD; Cuadrado, Maria J. MD, PhD; Hughes, Graham R. V. MD, FRCP; Khamashta, Munther A. MD, FRCP, PhD. The current study confirms that progression from primary APS to SLE or lupus-like disease is unusual, even after a long follow-up. Only 3 patients developed anti-dsDNA antibodies. The presence of a positive Coombs test might be a marker for the development of SLE in patients with primary APS. | ||||||||
| Lupus and Antiphospholipid Syndrome (APS) | ||||||||
| This article was published on Thursday 18 November, 2004. | ||||||||
| Lupus and Thrombosis | ||||||||
| September 2006. In summary, lupus patients are at significantly increased risk for premature atherosclerosis and thrombosis, which is a multifactorial process. A risk-stratified approach to thrombosis risk assessment (i.e., lupus disease activity/severity, traditional and lupus-related as well as acquired and genetic thrombotic risk factors, and aPL profile) is important in the management of lupus patients; however, there are no evidence-based recommendations yet for the primary thrombosis prevention. Current and future clinical lupus trials hopefully will further advance primary thrombosis prevention strategies. | ||||||||
| Non-invasive cardiac evaluation in patients with systemic lupus erythematosus. | ||||||||
| J Med. 2001;32(3-4):195-206. The aim of this study was non-invasive assessment of cardiac function in patients with systemic lupus erythematosus (SLE). In a group of 36 patients with SLE transthoracic echocardiography, standard ECG and the 24-hour ECG Holter monitoring were performed and the results were compared to a control group of 35 healthy volunteers. Significantly lower mean values of heart rate variability (HRV), the presence of late ventricular potentials and tendency to tachycardia were detected in SLE patients when compared to the control group. On echocardiography examination valvular lesions were found in 15 SLE patients but only in 5 of them were insignificant mitral or aortic regurgitant jets observed. Echocardiography did not reveal abnormalities in cardiac dimensions and left ventricle systolic function. Abnormal indexes of left ventricular filling were found in 3 patients. All SLE patients with antiphospholipid antibodies had some cardiovascular manifestation. | ||||||||
| Systemic Lupus Erythematosus and Antiphospholipid Antibodies | ||||||||
| Mountain States Regional Genetic Services Network Systemic Lupus Erythematosus and Antiphospholipid Antibodies http://www.obgyn.net/pb/articles/systemic_lupus_pg.htm 2002 | ||||||||
| Systemic Lupus Erythematosus and Antiphospholipid Antibodies: One Cause of Neurologic Dysfunction | ||||||||
| by Alan Lash, MD. This is an original article prepared for the Bay Area Lupus Foundation | ||||||||
| Systemic lupus erythematosus in a multiethnic US cohort (LUMINA): XXV. Smoking, older age, disease activity, lupus anticoagulant, and glucocorticoid dose as risk factors for the occurrence of venous thrombosis in lupus patients. | ||||||||
| Arthritis Rheum. 2005 Jul;52(7):2060-8 CONCLUSION: Venous thrombotic events occur early in the course of SLE. Our data confirm the association between LAC and venous thrombotic events. Smoking, shorter disease duration, older age, disease activity over time, and higher mean daily glucocorticoid dose were identified as additional risk factors for the development of this vascular complication. These findings may have implications for the management of patients with SLE. | ||||||||
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| APS - Lupus Research & Trials Information |
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| 12th International Congress on Anti-Phospholipid Antibodies | ||||||||
| 18-20 April 2007 in Florence, Italy | ||||||||
| A Pilot Study of a Cognitive Rehabilitation Intervention Program in Systemic Lupus Erythematosus (SLE) Patients | ||||||||
| Study of a novel group educational program for lupus patients to improve their ability to perform everyday tasks that have become difficult due to cognitive dysfunction. Sponsor: Mary Kirkland Center for Lupus Research. Principal Investigator: Melanie J. Harrison, MD, MS. HSS IRB #: 22076 | ||||||||
| A Study of the Genetics of Antiphospholipid Antibody Syndrome | ||||||||
| Written by: Melissa Hall | ||||||||
| Antiphospholipid Antibody Syndrome (APS) | ||||||||
| We know very little about why people develop APS. The goal of this research study is to identify genetic or environmental factors that may increase susceptibility to APS. We are looking for families where an individual has APS and one or more family members also have APS. We are also looking for families where an individual has APS and one or more family members has an autoimmune disease such as lupus, diabetes, rheumatoid arthritis, multiple sclerosis, or similar disorders. | ||||||||
| Antiphospholipid syndrome in patients with systemic lupus erythematosus treated by autologous hematopoietic stem cell transplantation | ||||||||
| Blood, 15 October 2005, Vol. 106, No. 8, pp. 2700-2709. Prepublished online as a Blood First Edition Paper on May 3, 2005; DOI 10.1182/blood-2005-01-0330. | ||||||||
| Antiphospholipid Syndrome Studies/Research | ||||||||
| One fast click to see the current studies dealing with APS. | ||||||||
| APLASA Study: Determining the Role of Aspirin for Thrombosis Prophylaxis in Antiphospholipid Syndrome | ||||||||
| An international multi-center, randomized, placebo (sugar tablet) controlled study designed to study whether low-dose aspirin can prevent blood clots in asymptomatic aPL-positive individuals (those who only have the antibodies but have never had a clot). Sponsors: Bayer Pharmaceuticals, Arthritis Foundation (New York Chapter). Principal Investigator: Doruk Erkan, MD. HSS Co-investigators: Melanie J. Harrison MD, MS, Michael D. Lockshin, MD, Lisa Sammaritano, MD, and Margaret Peterson, PhD. HSS IRB #: 20068 | ||||||||
| APLASA-P Study: Determining the Role of Aspirin in the Management of Antiphospholipid Syndrome Patients who Present Only with Pregnancy Morbidity | ||||||||
| An international multi-center, observational study designed to study whether low-dose aspirin can prevent blood clots in APS patients who have only had miscarriages but no blood clots in other vessels. Sponsors: Bayer Pharmaceuticals, Arthritis Foundation, New York Chapter. Principal Investigator: Doruk Erkan, MD. HSS Co-investigators: Melanie J. Harrison MD, MS, Michael D. Lockshin, MD, Lisa Sammaritano, MD, Margaret Peterson, PhD. HSS IRB #: 20069 | ||||||||
| APSCORE: National Antiphospholipid Syndrome Collaborative Registry | ||||||||
| National registry that collects clinical and laboratory information on patients with antiphospholipid antibodies and/or syndrome. Sponsor: National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS) Principal Investigator: Robert Roubey, MD (University of North Carolina at Chapel Hill) HSS Investigators: Michael D. Lockshin, MD and Lisa Sammaritano, MD. HSS Co-investigator: Doruk Erkan, MD. HSS IRB #: 20067 | ||||||||
| CAPS REGISTRY | ||||||||
| REGISTRY OF THE "EUROPEAN FORUM ON ANTIPHOSPHOLIPID ANTIBODIES" FOR PATIENTS WITH THE "CATASTROPHIC" ANTIPHOSPHOLIPID SYNDROME | ||||||||
| CDC Hemostatis and Thrombosis Center Pilot Site Locations | ||||||||
| The Centers for Disease Control and Prevention supports a network of specialized health-care centers to prevent and reduce complications experienced by persons with certain hereditary blood disorders. Currently, CDC has provided funds to eight "pilot" hemostatis and thrombosis centers to find out how to best provide treatment and preventative care to people with thrombosis or thrombophilia. These centers have multi-disciplinary teams of health-care specialists, state-of-the art clinical research programs, and provide outreach and education programs for patients. | ||||||||
| Clinical Trials Information for Patients | ||||||||
| In medical research, a clinical trial is an organized study conducted in people with a disease or condition to answer specific questions about a new treatment or a new way of using a known treatment. Each study tries to increase medical knowledge and to find new and better ways to help patients. Besides studying new drugs, clinical trials study new combinations of drugs already used in treatment, new ways of giving treatment, and how changes in lifestyle can help patients or prevent diseases from occurring. Other clinical trials compare the best known standard therapy with a newer therapy to see if one produces more cures and causes fewer side effects than the other. | ||||||||
| Clinical Trials.gov | ||||||||
| ClinicalTrials.gov provides regularly updated information about federally and privately supported clinical research in human volunteers. ClinicalTrials.gov gives you information about a trial's purpose, who may participate, locations, and phone numbers for more details. Before searching, you may want to learn more about clinical trials. | ||||||||
| Clinical Trials: Strokes | ||||||||
| Research will evaluate whether study medication will affect breathing, relax tightness in the muscles, and allow the wrist, fingers, and elbow to be straightened. Study will last for about 32 weeks; if you participate you will be asked to make nine visits to the study center, where you will have injections into your arm and hand. Research site located in Pittsburgh, Penn. | ||||||||
| Cognitive Function in Lupus and Primary Antiphospholipid Syndrome Patients over Time | ||||||||
| An ongoing, observational investigation of lupus patients designed to identify cognitive dysfunction and factors associated with it. Sponsors: Arthritis Foundation (New York Chapter and National Office), Mary Kirkland Center for Lupus Research, NIAMS. Principal Investigator: Melanie J. Harrison, MD, MS RCRC Co-investigators: Michael D. Lockshin, MD and Doruk Erkan, MD. HSS IRB #: 97054 | ||||||||
| CRISP (Computer Retrieval of Information on Scientific Projects) | ||||||||
| CRISP (Computer Retrieval of Information on Scientific Projects) is a searchable database of federally funded biomedical research projects conducted at universities, hospitals, and other research institutions. Maintained by the Office of Extramural Research at the National Institutes of Health (NIH), CRISP includes projects funded by the NIH as well as the Substance Abuse and Mental Health Services Administration (SAMHSA), Health Resources and Services Administration (HRSA), Food and Drug Administration (FDA), Centers for Disease Control and Prevention (CDC), Agency for Healthcare Research and Quality (AHRQ), and Office of Assistant Secretary of Health (OASH). Currently, 27 projects listed on CRISP involve APS. To read about these studies, click on the link below. In the 'enter search terms' box, enter 'antiphospholipid syndrome' and click the 'and' button below the box. Then click 'Submit Query.' | ||||||||
| Dexamethasone Treatment for Congenital Heart Block (CHB) in Newborns With Lupus | ||||||||
| Purpose: Some newborns are born with congenital heart block (CHB), a condition occurring in babies with neonatal lupus. The first part of the study will test the effectiveness of fluorinated steroids, including dexamethasone, in improving the heart function and general health of newborns who have auto-antibody-associated CHB. The second part of this study will use ultrasound and heart monitoring to observe high-risk pregnant women and their fetuses during the third trimester of pregnancy. | ||||||||
| Effect of antimalarials on thrombosis and survival in patients with systemic lupus erythematosus | ||||||||
| Lupus, Volume 15, Number 9, September 2006, pp. 577-583(7). Our study shows a protective effect of antimalarials against thrombosis and an increased survival of SLE patients taking these drugs. These data support the routine use of antimalarials in all patients with SLE. | ||||||||
| Genetics of antiphospholipid antibody syndrome (APS): Study #1 | ||||||||
| We know very little about what causes antiphospholipid antibody syndrome (APS). Sometimes several family members have APS. Other times, one person has APS and other relatives have a different autoimmune disease like lupus or diabetes. Because APS sometimes runs in families, we think that certain genes may cause APS. The purpose of this study is to find the genes that cause APS. | ||||||||
| Genetics of Antiphospholipid Antibody Syndrome Study 5806: Brochure | ||||||||
| We are looking for families where an individual has APS and one or more family members also have APS. We are also looking for families where an individual has APS and one or more family members has an autoimmune disease such as lupus, diabetes, rheumatoid arthritis, multiple sclerosis, or similar disorders. If your family has one or more members with APS or another autoimmune disorder, your family may be eligible to join the study. | ||||||||
| Genetics of Antiphospholipid Syndrome Study | ||||||||
| Written by: Thomas L Ortel, MD PhD. We are currently recruiting patients with APS for a study investigating the genetics of the disorder. The "proband" (typically the patient) must have symptoms associated with the disorder (such as blood clots, or recurrent miscarriages) and the presence of the antibodies associated with the syndrome (for example, the lupus anticoagulant, or anticardiolipin antibodies). The "proband" also needs to have at least one or more family members affected with APS, or one or more family members affected with another autoimmune disorder (for example, lupus, rheumatoid arthritis, multiple sclerosis, myasthenia gravis, etc.). We currently have participants enrolled from more than sixty families, and are looking for more! | ||||||||
| HUMAN GENOME SCIENCES ANNOUNCES FULL PRESENTATION OF RESULTS OF PHASE 2 CLINICAL TRIAL OF LYMPHOSTAT-B™ IN SYSTEMIC LUPUS ERYTHEMATOSUS | ||||||||
| Human Genome Sciences, Inc. announced that a Phase 2 clinical trial demonstrated that LymphoStat-B™ (belimumab) significantly reduced disease activity in patients with serologically active systemic lupus erythematosus (SLE), exhibited clinically relevant bioactivity, and was safe and well tolerated. Two oral presentations reported the complete study results today in Amsterdam at the Annual European Congress of Rheumatology (EULAR 2006). | ||||||||
| Identifying Unexplored Domains of Quality of Life in Lupus Patients | ||||||||
| Study to identify facets of daily life that have been impacted upon by lupus that are not routinely evaluated in studies of quality of life in adults with lupus. Sponsors: Mary Kirkland Center for Lupus Research. Principal Investigator: Melanie J. Harrison, MD, MS. HSS IRB #: 21067 | ||||||||
| Information for Potential Participants of the Genetics of Venous Thrombosis Study | ||||||||
| Why is this study being done? The purpose of this study is to determine inherited causes of venous thromboembolism(includes both deep vein thrombosis and pulmonary embolism) by studying families in whom two or more family members have had either deep vein thrombosis or pulmonary embolism. | ||||||||
| LCTC: Lupus Clinical Trials Consortium HSS Clinical Lupus Registry | ||||||||
| A clinical registry of systemic lupus erythematosus (SLE) patients that is created by the Department of Rheumatology at HSS for LCTC multicenter clinical lupus studies. Sponsor: Lupus Clinical Trials Consortium. Clinical Director: Doruk Erkan, MD. Center Director: Michael D. Lockshin, MD. HSS co-investigator: Melanie J. Harrison, MD. HSS IRB #: 24023 | ||||||||
| Longitudinal Study of the Clinical and Haematological Cause of Women With Antiphospholipid Antibodies. | ||||||||
| This study is currently recruiting patients. Verified by Imperial College London September 2005 | ||||||||
| Lupus Clinical Trials | ||||||||
| Our goal is to get as many people enrolled in lupus clinical trials as possible, so that the lupus community can finally get some answers on prevention, treatments, and a cure. | ||||||||
| Lupus Research Funding and Grants | ||||||||
| For information about lupus disease, including lupus symptoms and lupus treatment, visit the Alliance for Lupus Research. This voluntary health organization is committed to fighting and trying to cure lupus through medical research. | ||||||||
| Lupus Research Institute | ||||||||
| Recognizing that most major medical breakthroughs come from unexpected directions, the LRI fosters and supports only the highest ranked new science to prevent, treat and cure lupus. Millions in private sector funding support more than 50 scientists pursuing basic and clinical research studies at leading medical institutions around the country. | ||||||||
| Lupus Research Institute - Chicago | ||||||||
| The LRI Chicago shares the mission of its lead organization, the Lupus Research Institute, in championing only new science — the truly innovative research that will prevent, treat and cure lupus. | ||||||||
| Multiple Autoimmune Disease Genetics Consortium (MADGC) | ||||||||
| MADGC is a group of leading genetic researchers who have joined efforts to identify and understand the genes that autoimmune diseases have in common. This research may ultimately give doctors valuable knowledge about the basic causes of autoimmune disease and allow them to better diagnose and treat patients. | ||||||||
| NIH Launches Clinical Studies Nationwide to Investigate Rare Diseases | ||||||||
| $71 Million Effort to Address Neglected Conditions. The National Institutes of Health (NIH) announced today it is launching the first clinical studies of its Rare Diseases Clinical Research Network (RDCRN). More than 20 studies are expected to open in the next few months at about 50 sites across the United States and in several other countries including the United Kingdom, Japan, and Brazil. | ||||||||
| NIH Office of Rare Diseases | ||||||||
| Online resources and databases on completed current, or planned rare disease studies. If you enter the Web sites listed below, you will leave the ORD Web site. Please return to our Web site to find more information on rare diseases, patient support groups, and genetic testing laboratories and clinics. | ||||||||
| Pilot and Feasibility Study of Modafinil Treatment to Improve Cognitive Efficiency in SLE Patients | ||||||||
| This study is being conducted in order to determine if the FDA-approved drug Modafinil can improve cognitive function in patients with systemic lupus erythematosus (SLE). Sponsor: Mary Kirkland Center for Lupus Research. Principal Investigator: Melanie J. Harrison, MD, MS. Co-investigators: Michael D. Lockshin, MD. HSS IRB #: 25085 | ||||||||
| Predictors of Pregnancy Outcome in Systemic Lupus Erythematosus (SLE) and Antiphospholipid Syndrome (APS) | ||||||||
| This study is currently recruiting patients. Verified by Hospital for Special Surgery, New York September 2005 | ||||||||
| Pregnancy Complications & APS | ||||||||
| Antiphospholipid antibody syndrome (APS) is a medical problem that is associated with health risks for the pregnant mother and her baby. Initial selection criteria for the study include women between 18 and 44 years of age, diagnosis of antiphospholipid antibody syndrome and able to safely undergo treatment with heparin. Research site located in Chicago, Ill. | ||||||||
| PreventionGenetics | ||||||||
| PreventionGenetics performs DNA testing and DNA banking for health care and biomedical research. The mission of PreventionGenetics is to prevent disease and disability through genetic testing. PreventionGenetics is dedicated to the highest standards of genetic Ethical Principles. | ||||||||
| Quality of Life in Pediatric SLE | ||||||||
| Study to determine what characteristics of childhood lupus are associated with decreased quality of life. Sponsors: Hospital for Special Surgery, Department of Pediatrics Principal Investigator: Lakshimi Moorthy, MD and Thomas Lehman, MD. Co-investigators: Melanie J. Harrison, MD, MS, Margaret Peterson, PhD. | ||||||||
| Rare Diseases Clinical Research Network | ||||||||
| The Rare Diseases Clinical Research Network was created to facilitate collaboration among experts in many different types of rare diseases. Our goal is to contribute to the research and treatment of rare diseases by working together to identify biomarkers for disease risk, disease severity and activity, and clinical outcome, while also encouraging development of new approaches to diagnosis, prevention, and treatment. | ||||||||
| Rare Thrombotic Diseases Consortium | ||||||||
| The Duke Hemostasis & Thrombosis Center is a multidisciplinary program devoted to improving the care of patients with bleeding and clotting disorders through innovative basic and clinical research efforts. The Center was formed in 2001, when it was selected by the Centers for Disease Control & Prevention to be one of eight pilot Hemostasis & Thrombosis Centers in the United States . Leadership of the program includes faculty in Hematology, Maternal-Fetal Medicine, Pediatric Hematology, and Cardiology. Ongoing clinical research trials are investigating the optimal 'dose' of platelets for replacement therapy, the role of inherited hypercoagulable states in intrauterine growth restriction, and therapeutic strategies for patients with end-stage renal disease and recurrent vascular access occlusions. | ||||||||
| Rheumatology Clinical Research Center (RCRC) | ||||||||
| The RCRC at the Hospital for Special Surgery under the Division of Rheumatology is comprised of rheumatologists with strong interests and expertise in epidemiological, health services, and outcomes research in various rheumatic diseases. The goal of the RCRC is to design and execute scientifically sound clinical studies that are important to clinical rheumatology and the care of patients. | ||||||||
| Steroids and Antiphospholipid Syndrome- Related Pregnancy Loss | ||||||||
| This study is not yet open for patient recruitment. Verified by Imperial College London September 2005 | ||||||||
| Stroke Trials Directory | ||||||||
| Welcome to the Stroke Trials Directory, a continuously updated registry of randomized clinical trials. This project is a joint effort of the Internet Stroke Center at Washington University School of Medicine, the American Stroke Association and the National Institute of Neurological Disorders and Stroke. Please visit our frequently asked questions page for more information about this site. | ||||||||
| The Alliance for Lupus Research | ||||||||
| The Lupus Multiplex Registry and Repository (LMRR) | ||||||||
| The Lupus Multiplex Registry and Repository (LMRR) is: A long-term research project operating in conjunction with the Lupus Genetic Studies; Sponsored by the National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS, a branch of the National Institutes of Health or NIH); The only research resource of multiplex lupus families in the world that scientists in the USA can apply to use in their own lupus research; A collection of DE-IDENTIFIED materials from families with 2 or more members diagnosed with SLE including: Clinical Data, Serum, Plasma & DNA. | ||||||||
| The State of Lupus Research | ||||||||
| Thrombosis Interest Group of Canada (T.I.G.C) | ||||||||
| The Thrombosis Interest Group of Canada consists of a group of 40 specialists in fields related to thrombosis who collaborate to write evidence-based or consensus-based clinical guides on the investigation, management, and diagnosis of thrombotic disorders. | ||||||||
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| APS - Nephrology Related |
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| Acute renal failure due to bilateral renal artery thrombosis associated with primary antiphospholipid syndrome | ||||||||
| Nephrology Dialysis Transplantation, Vol 13, Issue 10 2645-2647, Copyright © 1998 by Oxford University Press | ||||||||
| Acute renal failure in a renal transplant donor due to primary antiphospholipid syndrome. | ||||||||
| Am J Nephrol. 2001 Jan-Feb;21(1):55-7. Primary antiphospholipid antibody (APA) syndrome, a common prothrombotic disorder, has been known in dialysis patients and renal transplant recipients. We report a case of primary APA syndrome presenting as a posttransplant complication in a renal transplant donor. A renal donor presented with acute, painless anuria due to renal artery thrombosis 6 years following renal transplant surgery, subsequent thrombosis of jugular catheter and arteriovenous fistula occurred, despite anticoagulation treatment, due to primary APA syndrome. This incident represents the most catastrophic complication reported in a renal donor due to primary APA syndrome. The validity of a prothrombotic assay in an organ donor workup to detect predilection to hypercoagulable disorders and to prevent such complications is open to question. The actual significance of APA in the blood is unclear; hence, the presence of APA in a potential renal donor would pose an ethical and practical dilemma. | ||||||||
| Antiphospholipid antibody syndrome and posttransplant renal thrombosis. | ||||||||
| Transplant Proc. 1999 Feb-Mar;31(1-2):230-3. | ||||||||
| Antiphospholipid syndrome and renal artery stenosis | ||||||||
| Q J Med 2000; 93: 127-129 © 2000 Association of Physicians | ||||||||
| Antiphospholipid syndrome and renal artery stenosis | ||||||||
| Q J Med 2000; 93: 127-129 © 2000 Association of Physicians. | ||||||||
| Antiphospholipid syndrome nephropathy in patients with systemic lupus erythematosus and antiphospholipid antibodies: prevalence, clinical associations, and long-term outcome. | ||||||||
| Arthritis Rheum. 2004 Aug;50(8):2569-79. OBJECTIVE: To evaluate the prevalence, clinical associations, and outcome of antiphospholipid syndrome (APS) nephropathy in patients with systemic lupus erythematosus (SLE) and antiphospholipid antibodies (aPL) and in SLE patients without aPL. METHODS: Kidney biopsy specimens obtained from 81 patients with aPL (18 of whom had APS) and 70 patients without aPL were retrospectively examined for the presence of APS nephropathy. Clinical and serologic data obtained at the time of kidney biopsy and during a mean followup of 7 years were recorded. In cases for which serial kidney biopsy specimens were available, the evolution of APS nephropathy was examined. RESULTS: APS nephropathy existed in 39.5% of patients with aPL, compared with only 4.3% of patients without aPL. APS nephropathy was associated with both lupus anticoagulant and anticardiolipin antibodies. Among aPL-positive SLE patients, APS nephropathy was found in two-thirds of those with APS and in one-third of those without APS. A strong association between APS nephropathy and the presence of arterial thrombosis and livedo reticularis was noted. Patients with APS nephropathy had a higher frequency of hypertension and elevated serum creatinine levels at the time of kidney biopsy but did not have a higher frequency of renal insufficiency, end-stage renal disease, or death at the end of followup. Serial kidney biopsy specimens were available from 11 patients and showed progression of APS nephropathy lesions. During followup, manifestations of APS (especially arterial thromboses) developed more frequently in the SLE/non-APS patients with APS nephropathy than in those without APS nephropathy. CONCLUSION: Among patients with SLE, APS nephropathy occurs almost exclusively in those with aPL, suggesting an important role of aPL in the pathogenesis of APS nephropathy. Patients with APS nephropathy develop hypertension, raised serum creatinine levels, and progression of histologic lesions, all of which are associated with a poor renal outcome. Manifestations of APS also tend to develop in these patients. APS nephropathy should be included in the APS classification criteria, and the use of appropriate anticoagulant therapy should be tested. | ||||||||
| Is it possible to diagnose primary anti-phospholipid syndrome (PAPS) on the basis of renal thrombotic microangiopathy (PAPS nephropathy) in the absence of other thrombotic process? | ||||||||
| Ren Fail. 2003 Nov;25(6):1043-9. | ||||||||
| Nephropathy and hypertension as manifestations in a 13-y-old girl with primary antiphospholipid syndrome. | ||||||||
| Acta Paediatr. 1998 Aug;87(8):903-7. | ||||||||
| Renal artery stenosis in hypertensive patients with antiphospholipid (Hughes) syndrome: outcome following anticoagulation. | ||||||||
| Rheumatology (Oxford). 2005 Mar;44(3):372-7. Epub 2004 Nov 30. | ||||||||
| Renal artery stenosis in the antiphospholipid (Hughes) syndrome and hypertension. | ||||||||
| Ann Rheum Dis. 2003 Oct;62(10):999-1002. CONCLUSION: A significantly increased prevalence of RAS (26%) was found in patients with APS and hypertension, compared with relatively young ( | ||||||||
| Renal infarction in a severely hypertensive patient with lupus erythematosus and antiphospholipid antibodies. | ||||||||
| Nephron. 1996;72(2):298-301. | ||||||||
| Renal infarction in antiphospholipid syndrome | ||||||||
| Conclusion: High index of suspicion is required in the appropriate clinical setting with unexplained asymmetrical functioning of kidney. | ||||||||
| Renal thrombotic microangiopathy in patients with systemic lupus erythematosus and the antiphospholipid syndrome. | ||||||||
| Am J Kidney Dis. 1992 Aug;20(2):150-8. | ||||||||
| Renal thrombotic microangiopathy in patients with systemic lupus erythematosus and the antiphospholipid syndrome. | ||||||||
| Am J Kidney Dis. 1992 Aug;20(2):150-8. Current studies indicate that a thrombotic microangiopathy (TMA) identifies patients with systemic lupus erythematosus (SLE) who are at high risk of progressing to end-stage renal disease. We have observed two patients with SLE and one patient with a primary antiphospholipid syndrome (APS) who developed acute renal insufficiency with thrombocytopenia. Renal biopsies showed a TMA characterized by thrombi or by cellular and mucoid intimal hyperplasia of small arteries and arterioles. No arterial or arteriolar immune-complex deposits were detected by immunofluorescent or electron microscopy. Biopsies from one SLE patient and the APS patient showed no immune-complex glomerular disease. Both had serum antiphospholipid antibodies (aPL). aPL were not detected in the serum of the other SLE patient who had an active lupus nephritis. Acute renal failure and thrombocytopenia resolved in each case following treatment by plasmapheresis or prednisone and heparin. None of the patients were initially treated with cytotoxic drugs. As more knowledge is gained, the accurate identification of renal vascular lesions in SLE or related diseases could influence renal prognosis and choice of therapy. The cases reported here provide further evidence that a TMA can cause acute renal failure independent of lupus nephritis. TMA should be distinguished from other forms of renal vascular disease, particularly a noninflammatory lupus microangiopathy, which is probably mediated by subendothelial immune-complex deposits. The absence of immunoglobulin deposits in vessels involved by a TMA indicates that microvascular thrombosis is promoted by mechanisms other than those usually attributed to immune-complex disease. Phospholipid reactive antibodies may be pathogenetic in some cases. | ||||||||
| Renovascular Hypertension Observed in a Patient With Antiphospholipid-Antibody Syndrome | ||||||||
| Japanese Circulation Journal Vol. 64 (2000) , No. 7 541-543 | ||||||||
| Study of renal histopathological correlation with anticardiolipin antibody status in patients with systemic lupus erythematosus | ||||||||
| Menon RN, Bichile LS. Study of renal histopathological correlation with anticardiolipin antibody status in patients with systemic lupus erythematosus. Indian J Nephrol 2007;17:53-60. Conclusions: Hypocomplementemia is the only probable marker of renal morbidity in the laboratory follow-up of the SLE patients. aCL positivity does contribute to greater incidence of interstitial fibrosis, tubular atrophy and capillary wall thickening in biopsy specimens. APSN is an independent entity that must be specifically sought for on histological assessment. | ||||||||
| The Intrarenal Vascular Lesions Associated with Primary Antiphospholipid Syndrome | ||||||||
| J Am Soc Nephrol 10:507-518, 1999 © 1999 American Society of Nephrology. This study allows us to approach the nephropathy of PAPS as an entity in its own right, characterized by small-vessel vaso-occlusive intrarenal pathology that likely evolves by repeated flares leading to a morphologic picture suggestive of its diagnosis even in the absence of history. This picture combines the lesions of TMA, FIH of the arteries and arterioles, and FCA. The clinical hallmark of this nephropathy is hypertension, only variably associated with renal insufficiency, protein-uria, or hematuria. Conversely, the recognition of such lesions on a biopsy performed for diagnostic purposes should lead to work-up for possible PAPS. However, we need to understand better the morbidity associated with this nephropathy to be able to define the indications for and modalities of treatment. | ||||||||
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| APS - Neurology Related |
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| Antiphospholipid Antibodies and Stroke in Young Women | ||||||||
| Stroke. 2002;33:2396. © 2002 American Heart Association, Inc. Conclusions— The results from this study support the importance of antiphospholipid antibodies as an independent risk factor for stroke in young women. | ||||||||
| Antiphospholipid Antibodies and Subsequent Thrombo-occlusive Events in Patients With Ischemic Stroke | ||||||||
| JAMA. 2004;291:576-584. | ||||||||
| Antiphospholipid antibodies in cerebral ischemia. | ||||||||
| Stroke. 1991 Jun;22(6):750-3. | ||||||||
| Antiphospholipid antibodies in young adults with stroke. | ||||||||
| J Thromb Thrombolysis. 2005 Oct;20(2):105-12. CONCLUSIONS: Antiphospholipid antibodies, particularly Lupus Anticoagulant, is an independent risk factor for first and possibly recurrent ischemic stroke in young adults. The best therapeutic strategy for preventing antiphospholipid antibody-associated recurrent stroke is not clear. | ||||||||
| ANTIPHOSPHOLIPID ANTIBODIES SYNDROME (APS) ASSOCIATED WITH HYPERHOMOCYSTEINEMIA RELATED TO MTHFR GENE C677T AND A1298C HETEROZYGOUS MUTATIONS IN A YOUNG MAN WITH IDIOPATHIC HYPOPARATHYROIDISM (DIGEORGE SYNDROME) | ||||||||
| Journal of Clinical Endocrinology & Metabolism 2006, 10.1210/jc.2005-2782. Conclusions: APS, revealed by anti-beta-2-glycoprotein (anti-2-GPI) and anti-prothrombin (anti-PT) antibodies positivity, and moderate HHcy related to heterozygous C677T and A1298C point mutations of the MTHFR gene, were identified as a possible cause of thrombotic disorder responsible for the widespread presence of cutaneous and cerebral lesions. | ||||||||
| Antiphospholipid antibodies syndrome in 'Stroke in young'. | ||||||||
| Mehndiratta MM, Bhattacharya A, Gupta M, Khawaja GK, Puri V. Antiphospholipid antibodies syndrome in 'Stroke in young'. Neurol India 1999;47:122-6 Promotes an INR of greater than 3.0. | ||||||||
| Antiphospholipid antibody syndrome manifested as a postoperative cerebrovascular event in a child. | ||||||||
| South Med J. 2000 Nov;93(11):1115-9. | ||||||||
| Antiphospholipid Antibody-Associated Chorea | ||||||||
| Conclusion. aPL-associated chorea occurs most often in women and severity is mild to moderate. Clinical expression of chorea does not differ between those with and without SLE. Anticoagulation should be reserved for thrombosis treatment and not simply for chorea in the presence of aPL, as 2 patients died of bleeding. The absence of IgM aCL in patients with APS supports prior evidence that IgG aCL and lupus anticoagulant may be the more clinically relevant antibodies for thrombosis. However, IgM aCL may be important in patients with chorea. (J Rheumatol First Release Oct 15 2008) | ||||||||
| Antiphospholipid syndrome and stroke | ||||||||
| Rinsho Shinkeigaku. 2005 Nov;45(11):852-5. Antiphospholipid syndrome is characterized by arterial or venous thrombosis, and the presence of antiphospholipid antibodies (aPL). APL are considered to be a cause of an acquired hypercoagulable state leading to stroke and transient ischemic attack (TIA). We examined the causes in 50 young patients with ischemic stroke. The most prevalent cause was atherosclerosis and the incidence of APS was 12.5%. APL comprise a heterogeneous group of autoantibodies, such as beta2-glycoprotein I dependent anticardiolipin antibody (beta2-GPIaCL), lupus anticoagulant (LA), and other antiphospholid-protein antibodies. We examined the incidence and the pathogenic role of antiphospholipid protein antibodies. The subjects comprised 250 patients (155 male, 95 females) with ischemic stroke, aged 26 to 92 years (mean 72 years). We measured beta2-GPI aCL, IgG aCL, LA, phosphatidyserine dependent antiprothrtombin antibody (PS-PT), antiphosphatidyl-serine antibody (PS), antiphosphatidyl-inositol antibody (PI) in each patient. The incidence of beta2-GPI aCL, IgG aCL, LA, phosphatidyserine, PS-PT, PS, and PI was 2.8%, 12%, 9.2%, 7.2%, 9.6%, and 8.8%, respectively. The incidence of young stroke patients under 50 years was 5.2%. Among 13 young stroke patients, 5 had SLE. Among 23 patients with LA., 18 (78%) patients had PS-PT. Anti-PS-PT antibody is closely related to LA. Antinuclear antibody was detected in 79% of the patients with aPS and/or aPI. We compared the carotid ultrasonographic findings in positive aPI or aPS patients with those in negative ones. Increased IMT, plaque score and carotid stenosis were more common in aPI and aPS-positive patients than in negative ones Three of 5 patients who showed positive beta2-GPI, aCL and LA, simulataneously, had sysyemic lupus erythematosus as an immulological background. Two of 3 patients with PI and/or PS and beta2-GPI and/or LA were patients with SLE. Antiphospholipid antibody was considered to be a risk factor of stroke, especially in SLE and/or young female patients. The incidence of lupus anticoagulant is more common than beta2-GPI aCL in ischemic stroke. In SLE patients with stroke, multi-antiphospholipid-protein antibodies was inclined to be present. LA is closely related to ant-PS-PT and aPI and aPS are associated with anti-nuclear antibody and precipitation of atherosclerosis. | ||||||||
| Antiphospholipid-Protein Antibodies and Ischemic Stroke | ||||||||
| (Stroke. 1998;29:1755-1758.) © 1998 American Heart Association, Inc. | ||||||||
| APS and MS | ||||||||
| Association of antiphospholipid antibodies with central nervous system disease in systemic lupus erythematosus. | ||||||||
| Am J Med. 1995 Oct;99(4):397-401. | ||||||||
| Brain vascular changes in the case of primary antiphospholipid syndrome. | ||||||||
| Folia Neuropathol. 1996;34(2):92-6. Morphological picture of arterial fibromuscular dysplasia (FMD) of carotid and cerebral arteries associated with intracranial aneurysm and thrombotic small vessel vasculopathy in a 34-year-old woman with primary antiphospholipid syndrome (PAPS) is presented. The patient died because of hemorrhage caused by aneurysm rupture. In the walls of the aneurysm and aneurysmal dilatation of middle cerebral artery dysplastic changes of FMD type were found. The case fulfills the clinical and serological criteria of antiphospholipid syndrome (APS). Microscopic examination of the brain showed occlusion of small cerebral vessels, characteristic for antiphospholipid syndrome. It was caused by fibrin thrombi and endothelial proliferation or fibrous webs in the vessel lumen. Neither features of systemic lupus erythematosus (SLE) nor related autoimmune diseases were observed in the morphological examination of the brain, skin and internal organs. Therefore, it was feasible to confirm the diagnosis of PAPS in the patient with FMD of the large cephalic arteries. | ||||||||
| Can neurologic manifestations of Hughes (antiphospholipid) syndrome be distinguished from multiple sclerosis? Analysis of 27 patients and review of the literature. | ||||||||
| Medicine (Baltimore). 2000 Jan;79(1):57-68. | ||||||||
| Central nervous system involvement in the antiphospholipid (Hughes) syndrome | ||||||||
| Rheumatology 2003; 42: 200-213. The antiphospholipid (Hughes) syndrome (APS) is characterized by arterial and/or venous thrombosis and pregnancy morbidity in the presence of anticardiolipin antibodies and/or lupus anticoagulant. APS can occur either as a primary disorder or secondary to a connective tissue disease, most frequently systemic lupus erythematosus. Central nervous system (CNS) involvement is one of the most prominent clinical manifestations of APS, and includes arterial and venous thrombotic events, psychiatric features and a variety of other non-thrombotic neurological syndromes. In this review we focus on the common and some of the less common CNS manifestations that have been reported in association with antiphospholipid antibodies. | ||||||||
| Cerebral microembolism, a disease marker for ischemic cerebrovascular events in the antiphospholipid syndrome of systemic lupus erythematosus? | ||||||||
| Acta Neurol Scand. 1999 Jun;99(6):356-61. CONCLUSION: MES may be related to disease activity in patients with SLE and APS. Their detection may help to assess individual cerebrovascular risk and contribute to therapeutic decision making and therapeutic monitoring. | ||||||||
| Cerebrovascular disease and antiphospholipid antibodies in systemic lupus erythematosus, lupus-like disease, and the primary antiphospholipid syndrome. | ||||||||
| Am J Med. 1989 Apr;86(4):391-9. | ||||||||
| Chorea and antiphospholipid antibodies: Treatment with methotrexate | ||||||||
| Neurology 2001;56:137-138. © 2001 American Academy of Neurology. | ||||||||
| Cognitive Deficits in Patients With Antiphospholipid Syndrome | ||||||||
| Arch Intern Med. 2006;166:2278-2284. Conclusions Cognitive deficits may often be found among patients with APS, independent of any history of central nervous system involvement. Livedo reticularis and the presence of white matter lesions on brain magnetic resonance imaging are associated with an increased risk for cognitive dysfunction in APS. | ||||||||
| Dementia associated with the antiphospholipid syndrome: clinical and radiological characteristics of 30 patients | ||||||||
| Rheumatology Advance Access originally published online on September 14, 2004. Rheumatology 2005 44(1):95-99; doi:10.1093/rheumatology/keh408. | ||||||||
| Factor V Leiden and Antiphospholipid Antibodies Are Significant Risk Factors for Ischemic Stroke in Children | ||||||||
| Stroke. 2000;31:1283. © 2000 American Heart Association, Inc. | ||||||||
| Features Associated with Epilepsy in the Antiphospholipid Syndrome | ||||||||
| J Rheumatol 2004;31:1344-8. Conclusion. Epilepsy is common in APS and most of the risk seems to be linked to vascular disease as manifested by extensive CNS involvement, valvulopathy, and livedo reticularis and to the presence of SLE. These factors, however, explain only part of the increased occurrence of epilepsy in APS and other causes such as direct immune interaction in the brain should be investigated. | ||||||||
| Hemidystonia symptomatic of primary antiphospholipid syndrome in childhood. | ||||||||
| Mov Disord. 1993 Jul;8(3):383-6. We suggest that PAPS must always be considered when isolated or recurrent focal cerebral ischaemia, and particularly hemidystonia, occur in childhood. | ||||||||
| Hypoperfusion of brain single photon emission computerized tomography in patients with antiphospholipid antibodies. | ||||||||
| J Dermatol Sci. 1997 Jan;14(1):20-8. Hypoperfusion areas might be caused by microarterial thrombosis, microvenous thrombosis or vascular spasms. Early detection of cerebral abnormalities allows steps to be taken to protect against irreversible progress of cerebral blood flow. Therefore, brain SPECT should be performed in patients with antiphospholipid antibodies. | ||||||||
| Low dose aspirin after ischemic stroke associated with antiphospholipid syndrome | ||||||||
| Neurology 2003;61:111-114 © 2003 American Academy of Neurology | ||||||||
| Management of the neurological manifestations of APS--what do the trials tell us? | ||||||||
| Thromb Res. 2004;114(5-6):489-99. CONCLUSIONS: (1) aPL are a risk factor for incident stroke (Grade A, established as useful for the given condition in the specified population). (2) The evidence to support the role of aPL in recurrent stroke is conflicting and, therefore, inconclusive. (3) Warfarin at moderate-intensity doses is equally effective in preventing a recurrent thrombotic event as warfarin at high-intensity doses in patients with APS (Grade A evidence, established as useful for the given condition in the specified population). (4) Warfarin, at moderate-intensity doses is as effective as aspirin (at a dose of 325 mg/day) in preventing recurrent thrombotic events in patients who are aPL-positive at the time of an initial stroke (Grade B evidence, probably useful for the given condition in the given population). (5) Currently there are no data to support the use of any prophylactic therapy in patients with aPL and no clinical manifestations for the purposes of preventing an incident stroke. | ||||||||
| Migraine in Hughes syndrome—heparin as a therapeutic trial? | ||||||||
| Q J Med 2001; 94: 114-115 © 2001 Association of Physicians | ||||||||
| Migraine, memory loss, and "multiple sclerosis ". Neurological features of the antiphospholipid (Hughes') syndrome | ||||||||
| Postgraduate Medical Journal 2003;79:81-83 © 2003 Fellowship of Postgraduate Medicine | ||||||||
| Multiple sclerosis, neuropsychiatric lupus and antiphospholipid syndrome: where do we stand? | ||||||||
| Rheumatology Advance Access originally published online on January 11, 2005 Rheumatology 2005 44(4):434-442; doi:10.1093/rheumatology/keh532 Rheumatology Vol. 44 No. 4 © British Society for Rheumatology 2005. A trial of anticoagulation might be worthwhile in some patients with atypical MS and consistently positive aPL. | ||||||||
| Neurological involvement as a poor prognostic factor in catastrophic antiphospholipid syndrome: autopsy findings in 12 cases | ||||||||
| Source: Lupus, Volume 12, Number 2, 1 February 2003, pp. 93-98(6) | ||||||||
| Neurometabolite Markers of Cerebral Injury in the Antiphospholipid Antibody Syndrome of Systemic Lupus Erythematosus | ||||||||
| Stroke. 1998;29:2254-2260. | ||||||||
| Recurrent acute transverse myelopathy: Association with antiphospholipid antibody syndrome | ||||||||
| Shaharao V, Bartakke S, Muranjan MN, Bavdekar MS, Bavdekar SB, Udani1 VP. Recurrent acute transverse myelopathy: Association with antiphospholipid antibody syndrome. Indian J Pediatr 2004;71:559-561 | ||||||||
| Some considerations about the possible pathological mechanisms at work in antiphospholipid syndrome and stroke | ||||||||
| Rev Neurol. 2003 Oct 1-15;37(7):654-7. INTRODUCTION AND DEVELOPMENT: Over the last two decades antiphospholipid syndrome (APS) has started to be recognized from the association of apparently anionic phospholipid-specific antibodies with thrombosis, thrombocytopenia and recurrent foetal losses. This syndrome affects patients with systemic lupus erythematosus and is considered to be an important cause of thromboembolic disease. Antiphospholipid antibodies are serum immunoglobulins that react with negatively charged phospholipids, albeit directly or by means of a cofactor, affect the coagulation system, and promote thrombosis. Recent research has been directed towards gaining an understanding of the mechanisms by which these antibodies are able to play a direct role in the pathophysiology of thrombosis, and the extent to which certain risk factors, such as smoking, high blood pressure, lipid disorders and so on, exert an influence over the expression of phospholipids in the cerebral endothelium. CONCLUSION: These antibodies have no single mechanism of action; different authors have described different pathological mechanisms, which help us to understand the heterogeneous clinical manifestations of APS. | ||||||||
| Stroke and antiphospholipid syndrome: the treatment debate | ||||||||
| Rheumatology Advance Access originally published online on April 19, 2005 Rheumatology 2005 44(8):971-974; doi:10.1093/rheumatology/keh666 | ||||||||
| The Antiphospholipid Syndrome and "Multiple Sclerosis" | ||||||||
| The blood disease that mimics MS - Hughes Syndrome | ||||||||
| How APS can mimic MS. | ||||||||
| Thrombotic cerebral arteriopathy in patients with the antiphospholipid syndrome. | ||||||||
| Mod Pathol. 1993 Nov;6(6):644-53. CONCLUSIONS: The cerebrovascular changes of the antiphospholipid syndrome are derived from a chronic thrombotic microangiopathy. The findings support the hypothesis that antiphospholipid antibodies can cause recurring episodes of intravascular thrombosis. | ||||||||
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| APS - Opthalmology Related |
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| A case of optic neuritis associated with anticardiolipin antibodies | ||||||||
| Rinsho Shinkeigaku. 1992 Mar;32(3):330-2. We reported a case of optic neuritis with the persistence of severe visual loss and central scotoma in a 26-year-old woman who was proven to have biologic false positive test for syphilis, and the elevated serum titres of IgG and IgM anticardiolipin antibodies. C.S.F. findings showed the absence of oligoclonal bands and the presence of IgM anticardiolipin antibody. She was treated twice at intervals of two weeks with methylprednisolone 1000 mg intravenously daily for three days (pulse therapy), and was started on oral prednisolone 60 mg daily which tapered gradually. After the second treatment of the pulse therapy, her visual acuity was improved remarkably and the titre of anticardiolipin antibodies became normal. Her clinical course seemed to be different from that of the optic neuritis of multiple sclerosis, in which many of patients recover near normal visual acuity after a first attack. We suggested that antiphospholipid antibodies might play a role in the etiology of her optic neuritis. | ||||||||
| Antiphospholipid Antibody Syndrome | ||||||||
| The most common ocular pathology is thrombosis with resultant ischemia. This manifests as central retinal vein or artery occlusion (CRVO or CRAO), anterior ischemic optic neuropathy (AION), transient ischemic attack (TIA), amaurosis fugax, isolated retinal hemorrhages and cotton wool spots, and retinal neovascularization. While these conditions typically occur in the elderly, APAS patients may experience them earlier in life. Besides the ocular manifestations, thrombi often affect other systems. Venous thromboses of the arm and leg, sagital, pelvic, mesenteric, portal and axillary vessels, and pulmonary embolism have all been encountered in APAS. With thrombosis in the arterial system, cerebrovascular accidents have also occurred. Thrombocytopenia (reduced platelet count) may also occur in these patients. | ||||||||
| Bilateral retinal vascular occlusion during antiphospholipid antibody syndrome: a case report | ||||||||
| J Fr Ophtalmol. 2005 May;28(5):503-7 DISCUSSION AND CONCLUSION: Central retinal artery or vein occlusion in a young patient must suggest the diagnosis of antiphospholipid antibody syndrome. The bilateralism of vascular occlusion is considered a severe factor because of its consequence on functional ocular and vital prognosis, where it can sound the alarm to the extension of thrombotic events to other vessels in the body. Antiphospholipid syndrome must be studied in cases of severe retinal vascular occlusion in young patients. Its diagnosis is important because the risk of recurrent thrombotic events may endanger functional and vital prognosis. | ||||||||
| Central retinal venous occlusion with co-existent thrombotic thrombocytopenic purpura and antiphospholipid syndrome | ||||||||
| British Journal of Ophthalmology 2003;87:658-659 © 2003 BMJ Publishing Group | ||||||||
| Handbook of Ocular Disease Management Antiphospholipid Syndrome | ||||||||
| Patients with antiphospholipid antibody syndrome (APAS) tend to be under age 50 and female. Up to 2% of women may have antiphospholipid antibodies. Many will also have lupus or other autoimmune diseases. | ||||||||
| Ocular symptoms in association with antiphospholipid antibodies. | ||||||||
| Graefes Arch Clin Exp Ophthalmol. 1998 Sep;236(9):658-68 CONCLUSIONS: We did not find a clear correlation between APA activity or the immunoglobulin classes in the individual and the severity of the ocular disease. The benefit from a therapy with the antiplatelet agent acetylsalicylic acid was evident in a reduction of the patients' transient visual disturbances and, in most cases, no further progression of permanent visual field defects was observed. | ||||||||
| Ophthalmic signs in antiphospholipid syndrome. Patient description | ||||||||
| Klin Oczna. 2004;106(4-5):661-3 CONCLUSIONS: presented characteristic general and ophthalmic signs point to APS. | ||||||||
| Primary antiphospholipid antibody syndrome and retinal occlusive vasculopathy. | ||||||||
| Am J Ophthalmol. 1997 Jun;123(6):848-50 CONCLUSIONS: In retinal vascular occlusions of unexplained origin, antiphospholipid antibodies may play an important role in the pathogenesis. Detecting these antibodies in the serum of patients with retinal vascular occlusion helps determine the appropriate treatment with long-term oral anticoagulants. | ||||||||
| Retinal vein occlusion in two patients with primary antiphospholipid syndrome. | ||||||||
| Korean J Intern Med. 2001 Dec;16(4):274-6 | ||||||||
| Review of severe vaso-occlusive retinopathy in systemic lupus erythematosus and the antiphospholipid syndrome: associations, visual outcomes, complications and treatment. | ||||||||
| Clin Experiment Ophthalmol. 2004 Feb;32(1):87-100 Conclusion: Severe vaso-occlusive retinopathy is a rare form of retinopathy in SLE often associated with poor visual prognosis and neovascularization. It may be a manifestation of the antiphospholipid syndrome. Treatment is aimed at preventing further thrombosis and complications arising from neovascularization. | ||||||||
| Sudden painless unilateral vision loss caused by branch retinal artery occlusion: implications for the primary care physician. | ||||||||
| Am J Med Sci. 2004 Jan;327(1):44-6 | ||||||||
| Visual disturbances and pathologic ocular findings in primary antiphospholipid syndrome. | ||||||||
| Ophthalmology. 1999 Aug;106(8):1537-40 CONCLUSIONS: Ocular involvement in PAS is uncommon. Transient visual disturbances are common, although most of them are related to central nervous system rather than ocular ischemia. Pathologic ophthalmic findings are unlikely to be found in asymptomatic patients or in patients with transient visual disturbances. Routine retinal fluoroangiography performed on patients with PAS is unproductive. | ||||||||
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| APS - Other |
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| A cross-sectional study of clinical thrombotic risk factors and preventive treatments in antiphospholipid syndrome | ||||||||
| Rheumatology 2002; 41: 924-929 © 2002 British Society for Rheumatology. Conclusion. While traditional risk factors were similar between groups, pregnancy and surgical procedures increased the risk of thrombosis. Hypertension and smoking were associated with arterial events. Possessing a combination of risk factors may increase the occurrence of arterial thrombosis but not venous thrombosis. Use of aspirin and/or hydroxychloroquine may be protective against thrombosis in asymptomatic aPL-positive individuals. | ||||||||
| Abdominal Thrombotic and Ischemic Manifestations of the Antiphospholipid Antibody Syndrome: CT Findings in 42 Patients1 | ||||||||
| Radiology. 2001;218:768-771. CONCLUSION: Patients who have circulating antiphospholipid antibodies are at risk for major abdominal vascular thromboses and organ infarction. Radiologists must be familiar with this syndrome; they may be the first physicians to suggest the diagnosis on the basis of findings of unusual or recurrent sites of thrombosis, especially in young patients. | ||||||||
| Adrenal failure followed by status epilepticus and hemolytic anemia in primary antiphospholipid syndrome | ||||||||
| Thrombosis Journal 2005, 3:6. doi:10.1186/1477-9560-3-6. Published: 18 April 2005. Conclusion: Adrenal failure is a rare complication of APS in children with only five cases reported to date. As shown in our patient, this syndrome can manifest in a diverse set of simultaneously occurring symptoms. | ||||||||
| An unusual cause of acute abdominal pain – A case presentation | ||||||||
| BMC Blood Disorders 2006, 6:1 doi:10.1186/1471-2326-6-1. Conclusion: This case presented to us as an acute abdominal pain. Subsequent investigations revealed the presence of splenic infarction. Coagulation risk factors for thrombosis proved negative. Haematological investigations revealed the presence of anticardiolipin antibodies at the first instance but subsequent determinations were negative. Hence, it mimicked Hughes syndrome initially but the criteria for temporal persistence of anticardiolipin antibody was not fulfilled. Unusual surgical presentation of a thrombotic abnormality as abdominal pain due to splenic infarction. | ||||||||
| Anti-phospholipid reactivity against cardiolipin metabolites occurring during endothelial cell apoptosis | ||||||||
| Arthritis Research & Therapy 2006, 8:R180 doi:10.1186/ar2091. We have recently shown that cardiolipin (CL) and its metabolites move from mitochondria to other cellular membranes during death receptor-mediated apoptosis. In this study we investigate the immunoreactivity to CL derivatives occurring during endothelial apoptosis in patients with antiphospholipid syndrome (APS) and systemic lupus erythematosus (SLE). We compared the serum immunoreactivity to CL to that of its derivatives monolysocardiolipin (MCL), dilysocardiolipin (DCL), and hydrocardiolipin (HCL), by means of both enzyme-linked immunosorbent assay and thin layer chromatography (TLC) immunostaining. In addition, we investigated the composition of phospholipid extracts from the plasma membrane of apoptotic endothelial cells and the binding of patients' sera to the surface of the same cells by using high performance TLC and immunofluorescence analysis. The average reactivity to MCL was comparable to that of CL and significantly higher than that for DCL and HCL in patients studied, both in the presence or in the absence of beta2-Glycoprotein I. Of relevance for the pathogenic role of these autoantibodies, IgG from patients' sera showed an increased focal reactivity with the plasma membrane of endothelial cells undergoing apoptosis. Interestingly, the phospholipid analysis of these light membrane fractions showed an accumulation of both CL and MCL. Our results demonstrated that a critical number of acyl chains in CL derivatives is important for the binding of anti-phospholipid antibodies and that MCL is an antigenic target with immunoreactivity comparable to CL in APS and SLE. Our finding also suggests a link between apoptotic perturbation of CL metabolism and the production of these antibodies. | ||||||||
| Antiphospholipid antibodies in patients with sensorineural hearing loss. | ||||||||
| Eur Arch Otorhinolaryngol. 2005 Aug;262(8):622-6. Sensorineural hearing loss can be associated with autoimmune diseases and the presence of antiphospholipid antibodies. Sixty patients (mean age 47 years, range 18-76 years) with sudden sensorineural hearing loss were studied with audiograms, stapedial thresholds, otoacoustic emissions, positional and caloric testing. The serologic testing included antibodies against phosphatidylserine and beta(2)-glycoprotein. Additionally, a group of 34 patients (mean age 65 years, range 31-81 years) with normal tension glaucoma was examined because in a previous study these patients were reported to have elevated concentrations of antiphospholipid antibodies with a coincidence of progressive sensorineural hearing loss. The baseline for antiphospholipid antibody levels was established in a control group of 40 healthy blood donors. In 12 of the 60 patients with sudden sensorineural hearing loss, levels of antiphospholipid antibodies were elevated. Antiphosphatidylserine IgM antibodies were significantly lower compared to controls and patients with the combination of hearing loss and normal tension glaucoma (Fisher's exact two-sided test, P < 0.01). Our data suggest that antibodies against beta2-glycoprotein seem to coincidence with an acute event, such as sudden sensorineural hearing loss, whereas antibodies against phosphatidylserine IgG are detectable in the prolonged sequel, such as in patients with progressive sensorineural hearing loss and normal tension glaucoma. | ||||||||
| Antiphospholipid antibody in localised scleroderma | ||||||||
| Annals of the Rheumatic Diseases 2003;62:771-774. Conclusions: These results suggest that aCL and LAC are the major autoantibodies in patients with generalised morphoea. | ||||||||
| Antiphospholipid antibody syndrome complicated by Grave's disease. | ||||||||
| J Dermatol. 2002 Dec;29(12):776-80. The report describes a woman with primary antiphospholipid antibody syndrome complicated with Grave's disease. Developing symptoms included a small cutaneous nodule on her finger and subsequently ecchymotic purpura on the cheeks, ears, buttocks and lower legs. Histological examinations showed thrombosed vessels in the dermis without or with hemorrhage, respectively. Laboratory investigation revealed positive lupus anticoagulant and immunogenic hyperthyroidism due to Grave's disease. There is a close relationship between the cutaneous manifestation of antiphospholipid antibody syndrome and the activities of Grave's disease and a possible link of antiphospholipid antibody syndrome with Grave's disease was suggested both by the etiology of the disease as well as the disease activity. | ||||||||
| Antiphospholipid antibody syndrome in a six-year-old female patient. | ||||||||
| Am J Ophthalmol. 2003 Apr;135(4):542-4 CONCLUSIONS: Although uncommon, retinovascular thrombosis in children can occur in APA syndrome. Testing for ACA and lupus anticoagulant should be considered. | ||||||||
| Antiphospholipid Antibody Syndrome Ulcers | ||||||||
| © 1996-2005, American College of Physicians. All rights reserved. | ||||||||
| Antiphospholipid antibody syndromes and the Internet. | ||||||||
| Lupus. 1996 Oct;5(5):418-24. Information regarding the antiphospholipid antibody syndrome is accessible on the Internet, and encompasses both physician specific and patient specific files. The quantity and quality of data available at these sites, however, varies greatly and both search-refining and data manipulating protocols and software need improvement. | ||||||||
| Antiphospholipid inner ear syndrome. | ||||||||
| Laryngoscope. 2005 May;115(5):879-83. CONCLUSIONS: These data support the hypothesis that antiphospholipid antibodies are involved in the pathogenesis of some forms of inner ear dysfunction, presumably by causing microthrombus formation in the labyrinthine vasculature. Basic science studies are required to better understand the mechanisms by which antiphospholipid antibodies mediate inner ear dysfunction. Clinical studies to evaluate the efficacy of anticoagulation in this group of patients are also required. | ||||||||
| Antiphospholipid Syndrome Has Many Faces | ||||||||
| 04/18/2002 By Anne MacLennan Review of Arthritis & Rheumatism Volume 46, Issue 4, 2002. Pages: 1019-1027. | ||||||||
| Asymptomatic sensorineural hearing loss in patients with systemic lupus erythematosus. | ||||||||
| J Clin Rheumatol. 2006 Oct;12(5):217-20. CONCLUSION: If it can be established how often this ASNHL progresses to a clinical problem, it can be important that, as part of initial studies, patients with SLE undergo audiometric tests. | ||||||||
| Colonic ulcers in propylthiouracil induced vasculitis with secondary antiphospholipid syndrome | ||||||||
| Postgraduate Medical Journal 2005;81:338-340. A 48 year old white woman was admitted to the hospital because of several bouts of migratory polyarthritis, weight loss, fever, and abdominal pain over a period of 15 months. She had been taking propylthiouracil 100 mg daily for three years for hyperthyroidism treatment. A test for antineutrophil cytoplasmic autoantibodies (ANCA) was positive with a perinuclear pattern of staining. Antiphospholipid antibodies were also detected. Colonoscopy showed several ulcers on intestinal mucosa and the biopsy specimen showed intense microscopic vasculitis. The patient is well after methylprednisolone pulse therapy and eight months of oral azathioprine. A surveillance colonoscopy showed complete healing of intestinal ulcers. No recurrence of symptoms has occurred and autoantibodies are negative, 10 months after treatment finished. The sequence of events suggests a propylthiouracil induced vasculitis p-ANCA positive and an antiphospholipid syndrome. This is the first report of colonic ulcers diagnosed and successfully treated in such circumstances. | ||||||||
| Endocrinologic manifestations of the antiphospholipid syndrome. | ||||||||
| Lupus. 2006;15(8):485-9. Clinicians should keep a high index of suspicion for adrenal insufficiency in patients with APS. | ||||||||
| Genomics and targeting antithrombotic therapy in antiphospholipid syndrome | ||||||||
| Blood, 15 February 2006, Vol. 107, No. 4, pp. 1249. | ||||||||
| HCV and HIV may trigger antiphospholipid syndrome | ||||||||
| Last Updated: 2004-04-16 12:25:03 -0400 (Reuters Health) | ||||||||
| Infectious origin of the antiphospholipid syndrome | ||||||||
| Annals of the Rheumatic Diseases 2006;65:2-6; doi:10.1136/ard.2005.045443 | ||||||||
| Influenza Vaccination in Patients with Antiphospholipid Antibodies | ||||||||
| Written by: Thomas L. Ortel, MD, PhD | ||||||||
| Ischemic Colitis due to Antiphospholipid Antibody Syndrome | ||||||||
| Comments: This 69 year old lady presented to the hospital with sudden onset of left lower quadrant pain and bloody diarrhea. She described having experienced similar episodes over the past 25 years occurring every several years but recently with increasing frequency. The episodes have typically lasted several days before resolving without sequella. A CT scan on this admission showed marked wall thickening of the distal transverse colon, the splenic flexure, and the descending colon. Inflammatory changes were seen in the surrounding pericolic fat. The wall thickening had a somewhat nonspecific appearance, although increased mucosal and serosal enhancement raised the possibility of ischemia. On the basis of this study, the differential diagnosis included either an infectious or an inflammatory process. Colonoscopy was then performed. The right and transverse colon was normal but the splenic flexure down to the sigmoid was diffusely abnormal with marked ulceration and mucosal edema. The broad segments of ulceration favored the diagnosis of an ischemic etiology and the prior history of periodic occurrence of these events made an infectious etiology less likely. Biopsies were performed and these showed focal ulceration with acute inflammatory exudates adjacent to intact mucosa. Other areas show crypt dropout, with regeneration seen in the remaining crypts and fibrin deposition in the lamina propria. These features were most suggestive of ischemic colitis. Cultures for C. diff and other pathogens were negative. At four week follow-up, complete healing was demonstrated in the affected areas. Colonic ischemia most frequently involves the splenic flexure and the left colon and can mimic inflammatory bowel disease or malignancy. While full thickness injury and acute perforation can occur, the mucosal changes usually resolve within 2 weeks. Stricture formation is rare. Usually no cause for the event can be determined. E coli O157 is a pathogen frequently associated with colonic ischemia. During her workup she was found to have an ANA positive at 1:2560 in a homogeneous and speckled pattern and a positive antiphospholipid antibody titer on two consecutive studies, thus establishing the diagnosis of antiphospholipid antibody syndrome as the cause of her recurrent ischemic events. Antiphospholipid antibody syndrome is caused by a class of antibodies which includes lupus anticoagulant and anti cardiolipin antibodies and appear to be directed against a spectrum of plasma proteins bound to phospholipids involved in the clotting cascade. Anti-phospholipid antibody syndrome more commonly is associated with pregnancy loss, recurrent CVAs, thrombophlebitis, pulmonary emboli and deep venous thrombosis. Prospective studies report rates of recurrent thromboembolic events in the range of 7-10% per patient per year. Anticoagulation therapy with warfarin reduces the rate of recurrent thrombotic events. Contributed by: Alexandra Gutierrez, M.D. G.I. Fellow Massachusetts General Hospital | ||||||||
| Management of antiphospholipid antibody syndrome: a systematic review. | ||||||||
| JAMA. 2006 Mar 1;295(9):1050-7. CONCLUSIONS: In patients with APS, moderate-intensity warfarin is effective for preventing recurrent venous thrombosis and perhaps also arterial thrombosis. Aspirin appears to be as effective as moderate-intensity warfarin for preventing recurrent stroke in patients with prior stroke and a single positive test result for antiphospholipid antibody. The optimal treatment of other thrombotic aspects of APS needs to be addressed in well-designed prospective studies. | ||||||||
| Neuroendocrine manifestations of phospholipid antibody disease identified by long-term follow-up study of patients with phospholipid antibodies. | ||||||||
| Ann N Y Acad Sci. 2006 Jun;1069:386-90. In conclusion: (1) Inflammatory disease may develop in some patients with aPL and appears to be set off by pregnancy, a known trigger for clinical thrombotic events in aPL patients. (2) Thyroid cancer may be associated with aPL, and this association warrants further study with larger number of patients. | ||||||||
| Nonthrombotic Manifestations of the Antiphospholipid Syndrome: Away from Thrombosis? | ||||||||
| © 2006. The Journal of Rheumatology Publishing Company Limited. | ||||||||
| Perioperative medical management of antiphospholipid syndrome: hospital for special surgery experience, review of literature, and recommendations. | ||||||||
| J Rheumatol. 2002 Apr;29(4):843-9. Patients with antiphospholipid syndrome (APS), who are predisposed to vascular thrombotic events, are at additional risk for thrombosis when they undergo surgery. Serious perioperative complications (recurrent thrombosis, catastrophic exacerbation, or bleeding) occur despite prophylaxis. We describe our perioperative experience with APS patients who underwent a variety of surgeries, review the literature, and discuss strategies that may guide other physicians in their perioperative evaluation and management of patients with APS. Recommendations: perioperative strategies should be clearly identified before surgical procedure; pharmacological and physical antithrombosis interventions vigorously employed; periods without anticoagulation kept to a minimum; and any deviation from a normal course should be considered a potential disease related event. | ||||||||
| Photocopy Machines and Occupational Antiphospholipid Syndrome | ||||||||
| IMAJ 2008;10:52–54. Two patients who worked for several years in the operation and maintenance of photocopy machines developed an autoimmune disease. In both, early manifestations were thromboembolic phenomena associated with anticardiolipin antibodies. Joint and kidney involvement emerged later, with the appearance of other autoantibodies. These two patients were occupationally exposed to ultraviolet irradiation, ozone emission, and possibly some oxides of heavy metals. To our knowledge this is the first report of occupational autoimmune disease in photocopy machine workers, and the first description of antiphospholipid syndrome as an occupational disease. The possible cause-effect inter-relationship between their occupational exposure and autoimmune disease is discussed. | ||||||||
| Spectrum of vascular pathology affecting patients with the antiphospholipid syndrome. | ||||||||
| Hum Pathol. 1995 Jul;26(7):716-24. A thrombotic microangiopathy that is identified in patients with the antiphospholipid syndrome (APS) represents only a part of the vascular pathology that can be associated with antiphospholipid antibodies (aPL). Tissues from two autopsies, four renal biopsies, two skin biopsies, and one amputated leg were obtained from six patients who met criteria for the diagnosis of APS. Three patients had systemic lupus erythematosus (SLE), one had lupus-like disease, and two had a primary APS. Five of the patients were hypertensive. Arteries of three patients disclosed fibrin thrombi along with widespread obstruction by recanalized intimal connective tissue. Small renal, leptomeningeal, and pulmonary arteries showed concentric cellular and fibrous intimal hyperplasia indistinguishable from hypertensive vascular disease. Glomerular capillary and afferent arteriolar thrombi were found in renal biopsies from two SLE patients. One of these SLE patients required a leg amputation in which the popliteal artery demonstrated thrombosis, intimal hyperplasia, and acute inflammation. The findings support clinical and experimental data that indicate aPLs cause thrombosis but suggest diversity in the pathogenetic mechanisms aPLs are capable of promoting. Inflammation seems to be rare and to accompany thrombosis. Intimal hyperplasia is particularly common. Its involvement of renal arteries may contribute to hypertension that develops in some APS patients. | ||||||||
| Spontaneous recovery of sudden sensorineural hearing loss: possible association with autoimmune disorders. | ||||||||
| J Am Acad Audiol. 2006 Jul-Aug;17(7):498-505. | ||||||||
| Superior mesenteric artery thrombosis associated with antiphospholipid syndrome. | ||||||||
| West J Med. 1991 August; 155(2): 174–176. | ||||||||
| The Antiphospholipid Story | ||||||||
| © 2003. The Journal of Rheumatology Publishing Company Limited. | ||||||||
| The systemic nature of the antiphospholipid syndrome. | ||||||||
| Scand J Rheumatol. 2004;33(6):365-72. Antiphospholipid syndrome (APS, Hughes' syndrome) is a systemic autoimmune disorder characterized by arterial and/or venous thrombosis and recurrent foetal loss, accompanied by mild to moderate thrombocytopaenia and elevated titres of antiphospholipid antibodies (aPLs): lupus anticoagulant (LAC) and/or anticardiolipin (aCL) antibodies. APS was defined originally in 1983 in systemic lupus erythematosus (SLE) patients, but later it was found that APS can be primary or secondary to other autoimmune diseases or malignancy. During the past 20 years many organs have been reported to be involved in this syndrome and the clinical manifestations are seen in every medical field. Moreover, many aPLs have been found in APS besides aCLs and LACs, which bind to the autoantigen beta-2-glycoprotein I (beta2GPI). Treatment for APS, based on antiplatelet and anticoagulation drugs, is dependent on various parameters, including whether SLE is also present, classical vs non-classical manifestations of the diseases, women with APS based on pregnancy morbidity, the presence of elevated aCL antibody titres in the absence of clinical manifestations, and catastrophic APS. | ||||||||
| Urologic damage of the primary antiphospholipid syndrome | ||||||||
| Arch Esp Urol. 2004 Sep;57(7):707-23. The antiphospholipid syndrome is an acquired autoimmune systemic disease generating a permanent hypercoagulability status with recurrent multiorgan thrombotic events due to circulating antiphospholipid antibodies. It may be secondary to a heterogeneous group of diseases (mainly lupus) and drugs, or primary if it appears isolated without any demonstrable systemic disease or concomitant medication. It is mainly characterized by venous or arterial recurrent thrombosis, recurrent abortion, thrombocytopenia, and circulating antiphospholipid auto-antibodies. Treatment with anticoagulants and correction of the hypercoagulable status contributing factors, arterial or venous thrombosis, and vascular risk aim to avoid new thrombosis episodes. Genitourynary system may be affected in any of its parts, generally by arterial or venous thrombosis. Kidney is the most frequently affected organ, in addition to transplanted kidney grafts, adrenal glands, bladder and testicles. There is a relationship between antiphospholipid syndrome and infertility. For the first time, we describe bladder involvement presenting as hyperreflexic neurogenic bladder with detrusor-sphincter dyssynergia after spontaneous spinal cord thrombosis in an asymptomatic adolescent with primary antiphospholipid syndrome which was unknown before. | ||||||||
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| APS - Pregnancy |
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| A Multicenter, Placebo-Controlled Pilot Study of Intravenous Immune Globulin Treatment of Antiphospholipid Syndrome During Pregnancy | ||||||||
| The American Journal of Obstetrics and Gynecology 01/01/2000 (Volume 182, Number 1) | ||||||||
| A study of sixty pregnancies in patients with the antiphospholipid syndrome. | ||||||||
| Clin Exp Rheumatol. 1996 Mar-Apr;14(2):131-6. | ||||||||
| Antiphospholipid antibodies and pregnancy loss | ||||||||
| Antiphospholipid antibodies and pregnancy rates and outcome in in vitro fertilization patients | ||||||||
| Authors: A. Denis, M. Guido, R. Adler, P. Bergh, C. Brenner, R. Scott, Jr. Source: Fertility and Sterility: June, 1997 (Vol. 67) Pages 1084-1090. | ||||||||
| Antiphospholipid Antibodies in Predicting Adverse Pregnancy Outcome | ||||||||
| Annals of Internal Medicine. 15 March 1994 | Volume 120 Issue 6 | Pages 470-475 | ||||||||
| Antiphospholipid Antibody Syndrome | ||||||||
| by Sara Marder, M.D. Instructor and Fellow in Maternal and Fetal Medicine Department of Obstetrics and Gynecology Yale University School of Medicine | ||||||||
| Antiphospholipid Antibody Syndrome (APS) or Hughes and Pregnancy | ||||||||
| 7/28/2003 | ||||||||
| Antiphospholipid Antibody Syndrome and Pregnancy Article by Stella Nowicki, DDS | ||||||||
| Background: Antiphospholipid syndrome (APS) is a recently recognized autoimmune condition that may manifest with fetal loss, thrombosis, or autoimmune thrombocytopenia. Women with these clinical features should be tested for lupus anticoagulant (LAC) and anticardiolipin (aCL) antibodies; most patients with APS have both LAC and aCL immunoglobulin G (IgG) antibodies. The diagnosis of APS requires the presence of both clinical and biological features. Systemic lupus erythematosus (SLE) is a chronic systemic disease with diverse clinical and laboratory manifestations. LAC (and aCL) predisposes to clotting in vivo, predominantly by interfering with the antithrombotic role of phospholipids (PLs); therefore, it is associated with clinical thrombosis, not bleeding. The antiphospholipid (aPL) autoantibodies bind moieties on negatively charged PLs or moieties formed by the interaction of negatively charged PLs with other lipids, PLs, or proteins. aPL antibodies belong to the large family of antibodies that react with negatively charged PLs, including cardiolipin, phosphatidylglycerol, phosphatidylinositol, phosphatidylserine, phosphatidylcholine, and phosphatidic acid. Last Updated: September 4, 2005 | ||||||||
| Antiphospholipid Syndrome (aPL) | ||||||||
| Because of the higher risks for stroke, pregnancy loss, and other complications with aPL, mothers need close monitoring of the disease. More frequent prenatal visits are often needed. | ||||||||
| Antiphospholipid Syndrome (aPL) | ||||||||
| Antiphospholipid syndrome is an autoimmune disease in which the body produces large amounts of antiphospholipid antibodies. Phospholipids are a special type of fat containing phosphate that makes up the outer walls of the body's cells. Antiphospholipid antibodies attack the phospholipids. This causes many different problems including increased blood clotting. Cardiolipin is one type of phospholipid and specific anticardiolipin antibodies may develop. | ||||||||
| Antiphospholipid syndrome and pregnancy | ||||||||
| Akush Ginekol (Sofiia). 2004;43(1):36-42. The antiphospholipid antibody syndrome (APLS) is multisystem, autoimmune disease, which is characterized by: thrombosis, obstetrics complications and thrombocytopenia. The two most clinically significant antiphospholipid antibodies (APLa) that are associated with recurrent pregnancy loss and thrombosis are anticardiolipin antibodies (ACL) and lupus anticoagulant (LA). The laboratory diagnosis is based on the presence of moderate to high positive ACL and/or LA. The inhibitory effect of antiphospholipid antibodies /APLa/ on trophoblast intercellular fusion, hormone production and invasion may cause pregnancy loss. Once placentation is established their thrombogenic action leads to decreased placental perfusion and subsequent infarction. The APLa--mediated inhibition of trophoblastic invasion and APLa--mediated vasculopathy in the placental bed arteries result in abnormal uterine artery /UA/ Doppler waveforms. The association between APLa and high resistance index /RI/ and/or diastolic notch /DN/ in the Doppler waveforms is high predictive for adverse pregnancy outcome, including pre-eclampsia/eclampsia, intrauterine growth retardation, placental abruption, intrauterine fetal death. Maternal treatment and careful monitoring of fetal well-being are mandatory in the management of these high-risk pregnancies. | ||||||||
| Antiphospholipid Syndrome in Pregnancy: A Randomized, Controlled Trial of Treatment | ||||||||
| Obstetrics & Gynecology 2002;100:408-413 © 2002 by The American College of Obstetricians and Gynecologists | ||||||||
| Antiphospholipid syndrome in pregnancy: a randomized, controlled trial of treatment. | ||||||||
| Obstet Gynecol 2002; 100:408-13. | ||||||||
| Clearance of Antiphospholipid Antibodies in Pregnancies Treated With Heparin | ||||||||
| Obstetrics & Gynecology 2001;97:394-398 © 2001 by The American College of Obstetricians and Gynecologists | ||||||||
| Management of thrombosis in antiphospholipid syndrome and systemic lupus erythematosus in pregnancy. | ||||||||
| Ann N Y Acad Sci. 2005 Jun;1051:606-12. Pregnancy is a high risk period for thrombosis in women with antiphospholipid syndrome (APS) and systemic lupus erythematosus (SLE) with antiphospholipid antibodies (aPL). Thrombosis may affect the mother, both in the venous and arterial beds, and also have a role in pregnancy loss. Thromboprophylaxis thus is warranted in most of these women. However, specific regimens containing low-dose aspirin, unfractionated heparin (UH), low molecular weight heparin (LMWH), and even dicumarinics in some circumstances after the first trimester are still a matter of controversy. Women with previous thrombosis should receive full antithrombotic doses of UH or LMWH during the whole pregnancy. Treatment of pregnancy losses is more debated, consisting of low-dose aspirin with or without associated heparin. The choice of treatment for a given patient must always take into account the woman's opinion after a careful discussion with the treating physician. Peripartum thromboprophylaxis with LMWH in women receiving aspirin-only regimens and prevention of osteoporosis in those treated with heparin are considered essential in the medical management of these patients. | ||||||||
| Obstetric Implications of Antiphospholipid Antibodies: Pregnancy Loss and Other Complications | ||||||||
| Clinical Obstetrics and Gynecology: Volume 44(1) March 2001 pp 2-10 | ||||||||
| Pregnancy Loss in the Antiphospholipid-Antibody Syndrome — A Possible Thrombogenic Mechanism | ||||||||
| N Engl J Med 1997;337 (154-160) | ||||||||
| TNF- Is a Critical Effector and a Target for Therapy in Antiphospholipid Antibody-Induced Pregnancy Loss | ||||||||
| The Journal of Immunology, 2005, 174: 485-490. The antiphospholipid syndrome (APS) is characterized by recurrent fetal loss, intrauterine growth restriction, and vascular thrombosis in the presence of antiphospholipid (aPL) Abs. Our studies in a murine model of APS induced by passive transfer of human aPL Abs have shown that activation of complement and recruitment of neutrophils into decidua are required for fetal loss, and emphasize the importance of inflammation in aPL Ab-induced pregnancy loss. In this study, we examine the role of TNF- in pregnancy complications associated with aPL Abs in a murine model of APS. We show that aPL Abs are specifically targeted to decidual tissue and cause a rapid increase in decidual and systemic TNF- levels. We identify the release of TNF- as a critical intermediate that acts downstream of C5 activation, based on the fetal protective effects of TNF- deficiency and TNF blockade and on the absence of increased TNF- levels in C5-deficient mice treated with aPL Abs. Our results suggest that TNF- links pathogenic aPL Abs to fetal damage and identify TNF blockade as a potential therapy for the pregnancy complications of APS. | ||||||||
| Use of the Low-Molecular-Weight Heparin Nadroparin During Pregnancy: A Review | ||||||||
| from Current Medical Research and Opinion Posted 04/02/2003. Summary: Antithrombotic therapy is often used during pregnancy for the treatment and prevention of venous thromboembolism, the prevention of systemic embolism in patients with heart valve prostheses and the prevention of foetal loss in patients with antiphospholipid syndrome. Low-molecular-weight heparins (LMWHs), including nadroparin, have largely replaced unfractionated heparin as the anticoagulant of choice. The use of the LMWH nadroparin in pregnant women at an increased risk of thromboembolism or foetal loss is discussed in this review. Deep vein thrombosis can be effectively treated or prevented with nadroparin without any serious adverse events. Nadroparin 0.1 ml/10 kg sc once daily prevents thromboembolic complications in pregnant women with heart valve prostheses. Nadroparin is also effective in preventing foetal loss, through contributing to normal placental development and in decreasing the risk of premature delivery in pregnant women with antiphospholipid syndrome or women with herpes and antiphospholipid syndrome. These results demonstrate nadroparin is effective, easy to administer and associated with a low incidence of foetal and maternal complications. The use of nadroparin at a prophylactic dose of 0.3 ml (2850 IU AXa, 95 IU/kg) (for high-risk patients, 0.3-0.6 ml) sc once daily, and a therapeutic dose of 0.1 ml/10 kg (95 IU/kg) sc twice daily, is in line with the latest international guidelines of the American College of Chest Physicians. | ||||||||
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| APS - Seronegative APS - SNAPS |
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| *SERONEGATIVE ANTIPHOSPHOLIPID ANTIBODY SYNDROME (SNAPS)…AND SNAPPING TO IT!! | ||||||||
| By: Gale McCarty, MD, FACR, FACP "You don't have the syndrome because your tests are low level or negative…" or "You have livedo, a heart valve problem, and thrombocytopenia, but these aren't listed as criteria for diagnosis" are comments made frequently by healthcare providers from many specialties to patients with clinical features suggesting the Antiphospholipid Antibody Syndrome (APS). | ||||||||
| Antiphospholipid syndrome without antiphospholipid antibodies at the time of the thrombotic event: transient 'seronegative' antiphospholipid syndrome? | ||||||||
| Clin Exp Rheumatol. 1997 Sep-Oct;15(5):541-4. The antiphospholipid syndrome (APS) is characterized by the presence of venous and arterial thrombosis, recurrent fetal losses and thrombocytopenia, associated with the presence of antiphospholipid antibodies (aPL). This syndrome may be "primary" or may be associated with other diseases, mainly systemic lupus erythematosus (SLE). However, some patients present the clinical picture of this syndrome but without evidence of aPL in their serum. The term "seronegative" APS has been proposed to categorize these patients. Here with we present two patients with seronegativity for aPL at the time of a thrombotic event, but in whom these antibodies were detected 2 and 7 months later. | ||||||||
| Pyoderma gangrenosum associated with the secondary antiphospholipid syndrome | ||||||||
| Seronegative antiphospholipid syndrome | ||||||||
| Annals of the Rheumatic Diseases 2003;62:1127 © 2003 by BMJ Publishing Group Ltd & European League Against Rheumatism | ||||||||
| Seronegative antiphospholipid syndrome | ||||||||
| Annals of the Rheumatic Diseases 2004;63:608 © 2004 by BMJ Publishing Group Ltd & European League Against Rheumatism | ||||||||
| Seronegative antiphospholipid syndrome associated with plasminogen activator inhibitor. | ||||||||
| Lupus. 1994 Jun;3(3):201-3. | ||||||||
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| APS Support Groups & Chat |
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| *APS Friends & Support Forum | ||||||||
| A forum run by Heidi & Tina founders of the APS Foundation of America, Inc., a non profit organization. This forum is an information source and a friendly support group for people who have Antiphospholipid Antibody Syndrome or for anyone who's lives are touched by it. It is sometimes referred to as APS, APLS, or APLA and is known as Hughes Syndrome or "Sticky Blood" in the UK. APS is associated with recurrent clotting events including premature stroke, repeated miscarriages, phlebitis, venous thrombosis and pulmonary thromboembolism. If this disease touches your life in some way, please feel free to join in our discussions! :) We're glad to have you visit! | ||||||||
| Antiphospholipid Antibody Syndrome | ||||||||
| This group is primarily intended for patients, caregivers, and physicians to foster discussions and share experiences related to APS. APLA are antibodies directed against certain phospholipids. Antiphospholipid syndrome is defined as the presence of antiphospholipid antibodies, arterial or venous thrombosis, recurrent spontaneous abortions, and thrombocytopenia. The syndrome can occur within the context of several diseases, mainly autoimmune, or it may be present without any recognizable disease, the so-called primary antiphospholipid syndrome. The APSFA does not endorse nor is affiliated with this group, we are just sharing it with you as a courtesy. The only support group that APSFA is affiliated with is APS Friends & Support Forum. It is worth noting that not all of the information presented comes from healthcare professionals. The APSFA does not have any control over the content of the this website and cannot confirm that all information provided by it is accurate. | ||||||||
| Antiphospholipid Antibody Syndrome | ||||||||
| A forum run by the APS Foundation of America, Inc., a non profit organization. This forum is an information source and a friendly support group for people who have Antiphospholipid Antibody Syndrome or for anyone who's lives are touched by it. It is sometimes referred to as APS, APLS, or APLA and is known as Hughes Syndrome or "Sticky Blood" in the UK. APS is associated with recurrent clotting events including premature stroke, repeated miscarriages, phlebitis, venous thrombosis and pulmonary thromboembolism. If this disease touches your life in some way, please feel free to join in our discussions! :) We're glad to have you visit! | ||||||||
| Antiphospholipid Syndrome Online Support Group | ||||||||
| A community of patients, family members and friends dedicated to dealing with Antiphospholipid Syndrome, together. The APSFA does not endorse nor is affiliated with this group, we are just sharing it with you as a courtesy. The only support group that APSFA is affiliated with is APS Friends & Support Forum. It is worth noting that not all of the information presented comes from healthcare professionals. The APSFA does not have any control over the content of the this website and cannot confirm that all information provided by it is accurate. | ||||||||
| APLSUK | ||||||||
| A group for people who have Hughes Syndrome / Antiphospholipid Syndrome. The APSFA does not endorse nor is affiliated with this group, we are just sharing it with you as a courtesy. The only support group that APSFA is affiliated with is APS Friends & Support Forum. It is worth noting that not all of the information presented comes from healthcare professionals. The APSFA does not have any control over the content of the this website and cannot confirm that all information provided by it is accurate. | ||||||||
| APS Foundation of America, Inc Facebook Fan Page | ||||||||
| A fan page & forum run by the founders of the APS Foundation of America, Inc., a non profit organization. This forum is an information source and a friendly support group for people who have Antiphospholipid Antibody Syndrome or for anyone who's lives are touched by it. It is sometimes referred to as APS, APLS, or APLA and is known as Hughes Syndrome or "Sticky Blood" in the UK. APS is associated with recurrent clotting events including premature stroke, repeated miscarriages, phlebitis, venous thrombosis and pulmonary thromboembolism. If this disease touches your life in some way, please feel free to join in our discussions! :) We're glad to have you visit! | ||||||||
| APS Foundation of America, Inc MySpace Fan Page | ||||||||
| A fan page & forum run by the founders of the APS Foundation of America, Inc., a non profit organization. This forum is an information source and a friendly support group for people who have Antiphospholipid Antibody Syndrome or for anyone who's lives are touched by it. It is sometimes referred to as APS, APLS, or APLA and is known as Hughes Syndrome or "Sticky Blood" in the UK. APS is associated with recurrent clotting events including premature stroke, repeated miscarriages, phlebitis, venous thrombosis and pulmonary thromboembolism. If this disease touches your life in some way, please feel free to join in our discussions! :) We're glad to have you visit! | ||||||||
| APS Message Board at Medicine.net | ||||||||
| Another small one, I believe free to join. The APSFA does not endorse nor is affiliated with this group, we are just sharing it with you as a courtesy. The only support group that APSFA is affiliated with is APS Friends & Support Forum. It is worth noting that not all of the information presented comes from healthcare professionals. The APSFA does not have any control over the content of the this website and cannot confirm that all information provided by it is accurate. | ||||||||
| APS or Antiphospholipid Antibody Syndrome | ||||||||
| Small and not so active, but free to join. Has alot of other forums as well. The APSFA does not endorse nor is affiliated with this group, we are just sharing it with you as a courtesy. The only support group that APSFA is affiliated with is APS Friends & Support Forum. It is worth noting that not all of the information presented comes from healthcare professionals. The APSFA does not have any control over the content of the this website and cannot confirm that all information provided by it is accurate. | ||||||||
| AutoImmunity Community | ||||||||
| AutoImmunity Community is a message board dedicated to the courageous people battling any of the over 60 autoimmune diseases, and is especially beneficial for those with multiple conditions. Friends and family are always welcome, as well as those who simply suspect they have an autoimmune disease. The APSFA does not endorse nor is affiliated with this group, we are just sharing it with you as a courtesy. The only support group that APSFA is affiliated with is APS Friends & Support Forum. It is worth noting that not all of the information presented comes from healthcare professionals. The APSFA does not have any control over the content of the this website and cannot confirm that all information provided by it is accurate. | ||||||||
| Blood Clot Survivors DVT/PE/APS | ||||||||
| A group of persons who have survived blood clots, strokes, PE's, or who have APS. The APSFA does not endorse nor is affiliated with this group, we are just sharing it with you as a courtesy. The only support group that APSFA is affiliated with is APS Friends & Support Forum. It is worth noting that not all of the information presented comes from healthcare professionals. The APSFA does not have any control over the content of the this website and cannot confirm that all information provided by it is accurate. | ||||||||
| Blood Clots Under 40 | ||||||||
| Welcome to the Blood Clot group. This is a group for younger people (or the young at heart!) to get support and advice if they are suffering from any form of blood clot, or if you are on Coumadin or Warfarin. The APSFA does not endorse nor is affiliated with this group, we are just sharing it with you as a courtesy. The only support group that APSFA is affiliated with is APS Friends & Support Forum. It is worth noting that not all of the information presented comes from healthcare professionals. The APSFA does not have any control over the content of the this website and cannot confirm that all information provided by it is accurate. | ||||||||
| Clotters Anonymous | ||||||||
| For general discussion of issues and health conditions surrounding blood clots, including, but not limited to deep vein thrombosis, pulmonary embolism, genetic clotting disorders and arterial clotting conditions such as atrial fibrillation and stroke. The APSFA does not endorse nor is affiliated with this group, we are just sharing it with you as a courtesy. The only support group that APSFA is affiliated with is APS Friends & Support Forum. It is worth noting that not all of the information presented comes from healthcare professionals. The APSFA does not have any control over the content of the this website and cannot confirm that all information provided by it is accurate. | ||||||||
| eHealth Forum | ||||||||
| Make sure you read the rules of this forum! The slightest step across their lines you are banned. The APSFA does not endorse nor is affiliated with this group, we are just sharing it with you as a courtesy. The only support group that APSFA is affiliated with is APS Friends & Support Forum. It is worth noting that not all of the information presented comes from healthcare professionals. The APSFA does not have any control over the content of the this website and cannot confirm that all information provided by it is accurate. | ||||||||
| Lupus Patients Understanding and Support | ||||||||
| Support Group and Forum. The APSFA does not endorse nor is affiliated with this group, we are just sharing it with you as a courtesy. The only support group that APSFA is affiliated with is APS Friends & Support Forum. It is worth noting that not all of the information presented comes from healthcare professionals. The APSFA does not have any control over the content of the this website and cannot confirm that all information provided by it is accurate. | ||||||||
| MySpace APS Group | ||||||||
| A group for people who have Hughes Syndrome / Antiphospholipid Syndrome. The APSFA does not endorse nor is affiliated with this group, we are just sharing it with you as a courtesy. The only support group that APSFA is affiliated with is APS Friends & Support Forum. It is worth noting that not all of the information presented comes from healthcare professionals. The APSFA does not have any control over the content of the this website and cannot confirm that all information provided by it is accurate. | ||||||||
| Pulmonary Embolism | ||||||||
| Suffering an episode of DVT or Pulmonary Embolism is a life threatening/changing event. Doctors are vague in sharing information about your prognosis or even your treatment. This group will serve as a place where people can compare their diagnosis and treatments and perhaps help each other in this scary journey!! The APSFA does not endorse nor is affiliated with this group, we are just sharing it with you as a courtesy. The only support group that APSFA is affiliated with is APS Friends & Support Forum. It is worth noting that not all of the information presented comes from healthcare professionals. The APSFA does not have any control over the content of the this website and cannot confirm that all information provided by it is accurate. | ||||||||
| Systemic APS Support Group | ||||||||
| This group is intended as a support group and a research resource for medical professionals, patients and families who wish to learn more about a little known lethal, yet treatable, blood disorder that causes hypercoagulation. This disease is known under many names and combinations of initials (APS, APLS, Catastrophic APS, Primary and secondary APS, Sero-Negative APS, Sticky Blood, as one of the connective tissue diseases and also by the non-descriptive name of Hughes Syndrome after one of the group of doctors who first described the disease). The APSFA does not endorse nor is affiliated with this group, we are just sharing it with you as a courtesy. The only support group that APSFA is affiliated with is APS Friends & Support Forum. It is worth noting that not all of the information presented comes from healthcare professionals. The APSFA does not have any control over the content of the this website and cannot confirm that all information provided by it is accurate. | ||||||||
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