Antiphospholipid antibody syndrome

The antiphospholipid antibody syndrome may develop both in patients with Systemic Lupus Erythematosus and as a primary disease, i.e. in the absence of both clinical manifestations and specific antibodies of Systemic Lupus Erythematosus. The primary syndrome is characterized by one of the following clinical manifestations: recurrent thromboembolic events, spontaneous abortions, immune thrombocytopenia, associated with laboratory signs such as the presence of anticardiolipin antibodies, lupus anticoagulant and/or antiphospholipid antibodies. Thrombosis by antiphospholipid antibodies is due to endothelial damage rather than to vasculitis. Several mechanisms have been postulated to explain the thrombotic effect of these antibodies. Anticoagulants, both dicumarol and heparin, associated with prednisone, are the classic therapy. High doses of immunoglobulins, plasmapheresis, immunoadsorbent plasmapheresis have also been used. Corticosteroids in bolus, possibly associated with cyclophosphamide also in bolus and plasmapheresis can be tried in more severe cases.