Anti-phospholipid Antibody Syndrome or Hugh's syndrome is a heterogeneous disorder, first fully described in 1980s. The syndrome is caused by the presence of specific
antibodies against
phospholipid binding
plasma proteins in the serum of the patient, with or without underlying
autoimmune diseases, that causes prolongation of tests of coagulation. High index of clinical suspicion is required for diagnosis of
Anti-phospholipid Antibody Syndrome.
Stroke or
myocardial infarction in young, unprovoked recurrent
deep vein thrombosis and recurrent pregnancy loss are typical scenarios where
Anti-Phospholipid Antibody Syndrome should be suspected. Presence of non-criteria manifestations like
livedo reticularis,
skin ulcers, nephropathy,
valvular heart disease and
thrombocytopenia adds to diagnostic clue for presence of
Anti-Phospholipid Antibody Syndrome. Treatment of
Anti-Phospholipid Antibody Syndrome has preventive and therapeutic aspects that usually focus on thrombotic and obstetric manifestations of the disease. Therapeutic anti-coagulation with
heparin followed by
warfarin is required for patients presenting with acute
thrombosis. Those with
venous thrombosis are given moderate intensity
warfarin International Normalized Ratio, 2-3), whereas those with arterial
thrombosis or recurrent
venous thrombosis even on
warfarin are treated with high intensity
warfarin (International Normalized Ratio, 3-4). Similarly, anticoagulation with
heparin is advised in patients with obstetric
Anti-Phospholipid Antibody Syndrome throughout pregnancy and up to six weeks postpartum. Treatment recommendations are still not clear for asymptomatic
Anti-Phospholipid Antibody Syndrome positive patients and in those with non-criteria manifestations of the disease.
Steroids,
intravenous immunoglobulin and
immunosuppressant are reported to be effective in severe cases of catastrophic antiphospholid syndrome 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. Keywords:
anticoagulants;
anti-phospholipid syndrome; obstetric APS; thrombotic APS.