Antiphospholipid syndrome.

PURPOSE OF REVIEW: To review the recently published studies that can guide physicians in the management of persistently antiphospholipid antibody (aPL)-positive patients.

RECENT FINDINGS: Two recent prospective randomized controlled trials of two intensities of warfarin concluded that both moderate and high-intensity anticoagulation are similarly protective in antiphospholipid syndrome patients after the first thrombosis. Despite lack of controlled studies, there is experimental evidence that hydroxychloroquine and statins may play a role in the management of aPL-positive patients. In the mouse model of antiphospholipid syndrome that involves the injection of high-titer antiphospholipid syndrome human serum, complement activation is essential in aPL-mediated fetal loss and heparin prevents aPL-induced complement activation.

SUMMARY: Primary thrombosis prevention in persistently aPL-positive individuals lacks an evidence-based approach; elimination of reversible thrombosis risk factors and prophylaxis during high-risk periods is crucial. Secondary thrombosis prevention in persistently aPL-positive individuals lacks a risk-stratified approach; although the current recommendation is life-long warfarin, the necessity, duration, and the intensity of warfarin are still debated. Catastrophic antiphospholipid syndrome patients usually receive a combination of anticoagulation, corticosteroids, intravenous immunoglobulin, and plasma exchange; there is a clear need to test new agents. A common strategy to prevent fetal loss in aPL-positive patients with history of pregnancy morbidities is low-dose aspirin and heparin; if patients fail this regimen, the next step is the addition of intravenous immunoglobulin although this is not supported by controlled studies. Currently, there is no evidence that anticoagulation is effective for nonthrombotic manifestations of antiphospholipid antibodies.