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Maintenance of blood flow involves the dynamic interactions between the endothelium, circulating cellular components, coagulation factors, and factors involved in fibrinolysis. Autoantibodies, inflammatory cytokines and other undefined triggers in a genetically predisposed person may lead to an imbalance in the equilibrium between the various hemostatic pathways resulting in potentially catastrophic thrombotic events. This article reviews the clinical manifestations and treatment of selected, potentially life-threatening thrombotic syndromes that may occur in association with autoimmune diseases. Special mention is made of thrombotic events associated with Behçet's syndrome.

Deterioration in the function of major organs in patients with systemic lupus erythematosus may be due to causes directly related to the disease itself or to non-lupus related causes. In patients with rapidly progressive immune-mediated disease, therapy may be initiated with pulse glucocorticoids alone or in combination with pulse cyclophosphamide. For selected patients with aggressive life-threatening disease or patients refractory to treatment, experimental therapies such as plasmapheresis or intravenous immunoglobulin may be used as adjuncts in their management.