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Antiphospholipid syndrome: A review

Antiphospholipid syndrome is being increasingly recognized as a disease with a myriad of clinical manifestations ranging from recurrent thrombosis and pregnancy morbidity to valvular lesions, transverse myelitis, thrombocytopenia and hemolytic anemia. It may be primary or secondary, i.e., associated with other autoimmune diseases. The latest classification criteria (Sydney 2006) recognize just three tests to define this syndrome-lupus anticoagulant, anticardiolipin antobodies and anti β2 glycoprotein 1 antibodies. Treatment of thrombotic events involves lifelong anticoagulation with vitamin K antagonists like warfarin. Antiphospholipid antibody syndrome (APS) with only pregnancy morbidity is treated with thromboprophylaxis using heparin during pregnancy and postpartum for 6 weeks. Catastrophic APS occurs in approximately 1% of APS, and is characterized by microvascular thrombosis (thrombotic storm) and organ dysfunction. In this review we discuss the pathogenesis, diagnosis, treatment and prognosis of the APS.