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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 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

Lupus and Antiphospholipid Syndrome (APS)

This article was published on Thursday 18 November, 2004.

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.

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)

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

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.

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

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

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.

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.

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.

Association between antiphospholipid antibodies and epilepsy in patients with systemic lupus erythematosus.

Arthritis Rheum. 1994 Apr;37(4):568-71.

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.

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.

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.

Systemic Lupus Erythematosus and Antiphospholipid Antibodies

Mountain States Regional Genetic Services Network Systemic Lupus Erythematosus and Antiphospholipid Antibodies 2002

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 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.

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.

Antiphospholipid antibody syndrome and polymyalgia rheumatica/giant cell arteritis

Rheumatology 2000; 39: 565-567 © 2000 British Society for Rheumatology

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


Michelle Petri, M.D., M.P.H. Associate Professor of Medicine The Johns Hopkins University School of Medicine Baltimore, MD

Autoantibodies to human prothrombin and clinical manifestations in 207 patients with systemic lupus erythematosus.

J Rheumatol. 1998 Jun;25(6):1104-8.

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.

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