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Catastrophic Antiphospholipid Syndrome (CAPS)

The catastrophic antiphospholipid syndrome (CAPS) is a very rare complication encountered in a subset of patients with antiphospholipid antibody syndrome. This rare syndrome is characterized by the development of multiple blood clots that block small blood vessels in several organs in the body. The organs most commonly affected by these small blood clots include the heart, lungs, nervous system, and kidneys. In many ways, this syndrome is similar to another rare disease, thrombotic thrombocytopenic purpura.

Many patients who develop this rare complication have lupus, and infections have been reported to potentially increase a patient's risk to develop the syndrome. Even with the best treatment, as many as half the patients who develop this syndrome do not survive.

Treatment includes anticoagulation (blood thinners), steroids, and a procedure called 'plasma exchange'. Plasma exchange refers to a process whereby a patient's plasma (the liquid part of the blood) is removed and replaced with plasma from blood donors. Patients who survive this life-threatening complication are generally maintained on long-term anticoagulant therapy.

Thrombotic Storm

Thrombotic storm is characterized by the rapid development of multiple thromboses (blood clots) in a patient with an underlying hypercoagulable state. The catastrophic antiphospholipid syndrome is one type of thrombotic storm, but patients may have other risk factors for forming blood clots. Treatment is similar to the approach used for patients with catastrophic antiphospholipid syndrome.

The above information is located at: http://rarediseasesnetwork.epi.usf.edu/rtdc/learnmore/index.htm#storm

Reproduced with permission by the Rare Thrombotic Diseases Consortium.

Other Catastrophic Antiphospholipid Syndrome (CAPS) Information Resources:

Catastrophic Antiphospholipid Syndrome (CAPS)
(http://www.thedoctorwillseeyounow.com/articles/arthritis/caps_9/)
Juan Javier Lichauco, M.D., Jayashree Sinha, M.D.,, and Peter Barland, M.D. February 2002

Catastrophic Antiphospholipid Syndrome
(http://jic.sagepub.com/cgi/reprint/21/3/144) (registration required)
Journal of Intensive Care Medicine, Vol. 21, No. 3, 144-159 (2006). DOI: 10.1177/0885066606287041. © 2006 SAGE Publications

Long term outcome of catastrophic antiphospholipid syndrome survivors
(http://ard.bmjjournals.com/cgi/content/full/62/6/530) (registration required)
Annals of the Rheumatic Diseases 2003;62:530-533 © 2003 by BMJ Publishing Group & European League Against Rheumatism. 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
(http://www.lupus.org/webmodules/articles/ispviewer.asp?a=438&print=yes)
(2006) 2, 81-89 - Nature Clinical Practice Rheumatology. Catastrophic antiphospholipid syndrome, a severe form of antiphospholipid syndrome, is associated with a high mortality rate; approximately 50% of patients die from thrombotic diathesis. As discussed in this Review, efforts to facilitate early diagnosis, institute effective treatments in a timely manner, and better understand the causes of this extreme condition are needed to improve patient outcome.

Page updated on: 01/07/08

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