A Simplified Understanding of the Black Swan: Anti-phospholipid Antibody Syndrome

Abstract

Anti-phospholipid antibody syndrome is caused by the presence of specific antibodies against phospholipid-binding plasma proteins in serum of patient, with or without underlying autoimmune diseases, that causes prolongation of tests of coagulation. High index of clinical suspicion is required for diagnosis. Stroke or myocardial infarction in young, unprovoked recurrent deep vein thrombosis and recurrent pregnancy loss are typical scenarios where it should be suspected. Presence of non-criteria manifestations like livedoreticularis, skin ulcers, nephropathy, valvular-heart disease and thrombocytopenia adds to diagnostic clue for presence of the syndrome. Therapeutic anti-coagulation with heparin followed by warfarin is required for patients with acute thrombosis. Those with venous thrombosis are given moderate-intensity warfarin(INR 2-3), whereas those with arterial thrombosis or recurrent venous thrombosis even on warfarin are treated with high intensity warfarin (INR 3-4). Similarly, anticoagulation with heparin is advised throughout pregnancy and up to six weeks postpartum. Treatment recommendations are still not clear for asymptomatic patients and in those with non-criteria manifestations of the disease. Steroids, intravenous immunoglobulin and immunosuppressant are reported to be effective in catastrophic cases characterized by rapid small vessel thrombotic involvement of multiple organ systems. Studies are evaluating the efficacy of direct thrombin inhibitors in the management of refractory cases.