Antiphospholipid Syndrome (APS) - An Update on Clinical Features and Treatment Options

Antiphospholipid syndrome (APS) is an autoantibody disorder characterized by the presence of antiphospholipid (APL) antibodies and heterogeneous clinical manifestations. Patients may present with recurrent thrombosis, obstetric morbidity, cardiac valvular lesions, thrombocytopenia, skin lesions, renal or neurologic abnormalities. We provide a comprehensive review of these diverse clinical features except renal and obstetric complications. Treatment of APS can be challenging as one tries to balance the benefit of anticoagulation therapy in this hypercoagulable state while minimizing the risk of bleeding. We discuss the various therapeutic options including the role of aspirin, warfarin, low molecular weight heparin, new direct thrombin inhibitors, hydroxychloroquine, intravenous gamma globulin, rituximab and others. Lower risk APS patients (i.e. first venous thrombosis) should receive warfarin with a target INR of 2.0-3.0. Higher risk patients (i.e. arterial thrombosis or recurrent venous events) have a target INR of >3.0. Currently, warfarin remains the mainstay in treatment of APS. Because of lack of adequate data, the newer oral direct inhibitors should be considered only when there is a known allergy/ intolerance or poor control with warfarin. Additional vascular and thrombotic risk factors should be aggressively reduced. Further studies involving large number of APS patients, diagnosed according to accepted criteria, are needed to better define the role of newer anticoagulants and other novel therapies.