Systemic lupus erythematosus (SLE) is a complex autoimmune disease with variable clinical manifestations that is characterized by flares and periods of relative quiescence. The disease occurs approximately 10 times more frequently in women and is more prevalent among certain ethnic groups. The etiology is complex and dependent upon an interaction of genetic, hormonal, and environmental factors. Corticosteroids and immunosuppressive agents have transformed the outlook for patients with lupus. Unfortunately, the increased lifespan unmasked an accelerated process of atherosclerosis and cardiovascular disease. Early mortality is usually attributable to active lupus, but deaths late in the disease process are often secondary to thrombotic events. Advancements in the understanding of molecular and cellular mechanisms involved in the pathogenesis have resulted in development of novel therapies. Immunomodulatory drugs developed for other diseases are being investigated for use in specific manifestations of lupus. Individualization of treatment and lifelong monitoring are required in most patients.
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