Management of antiphospholipid syndrome

Abstract

Antiphospholipid syndrome (APS) is an autoimmune disease characterised by vascular thrombosis and/or pregnancy morbidity in the presence of persistently positive serum tests for antiphospholipid antibodies. Management of APS centres on preventing these clinical events and in preventing chronic damage caused by these events. In patients with thrombotic APS, long-term anticoagulation is recommended in the majority of cases. Although there were hopes that direct-acting oral anticoagulants could replace warfarin for prevention of thrombosis in patients with APS, this now seems less likely due to recent trial results. There is no evidence for use of anticoagulation in people who are aPL-positive but have never had a thrombosis but low-dose aspirin may be beneficial in those who have a higher-risk aPL profile. Management of obstetric APS is with daily subcutaneous heparin and low-dose aspirin. This gives a live birth rate of 70% or more. Catastrophic APS is rare, occurring in 1% of patients with APS. It is characterised by thrombosis in multiple organs simultaneously, with a high mortality rate. The management is with corticosteroids, anticoagulation, and immunoglobulins or plasma exchange.