Catastrophic antiphospholipid syndrome: is life-long anticoagulation therapy required?

Catastrophic antiphospholipid syndrome (CAPS) is an unusual complication of antiphospholipid syndrome (APS) occurring in about 1% of patients. If left untreated, mortality can be as high as 50%. Therapy of APS and its complication CAPS is hampered by the lack of validated prospective, controlled, intervention clinical trials, although there is consensus that treatment should include anticoagulation therapy. But there are issues that need to be addressed such as duration and intensity of therapy. The present report describes our experience in 7 patients with CAPS in whom anticoagulation was discontinued after 6 months of therapy. During an average follow-up of 5.5 years, only 2 patients exhibited one episode each of recurrent venous thrombosis, but none of the patients in whom anticoagulation was discontinued experienced recurrent CAPS.Key Points• Discontinuation of long-term anticoagulation therapy in CAPS patients was not followed by recurrence of CAPS.