BEHCETS-DISEASE

Objectives: To review the new data on the epidemiology, etiopathogenes is, clinicolaboratory spectrum, prognosis, and treatments of Behcet's disease (ED). Methods: The information concerning the etiopathogenesis of the disease is divided into infection, immune, and genetic factors . The clinical features of the disease are discussed according to the organ or system involved. Treatment is described as general, local, an d systemic. Results: ED is a multisystem vasculitis with recurrent sym ptoms. It affects mainly people living around the Mediterranean basin and in Japan. The mean age at onset is the third decade. Children are rarely affected, and few neonatal cases have been reported. In large s eries of patients, men predominate over women. Infectious agents, immu ne mechanisms, and genetic factors are implicated in the etiopathogene sis of the disease, which remains to be elucidated. The pathology of t he lesions consists of widespread vasculitis. Eyes, skin, joints, the oral cavity, blood vessels, and central nervous system are usually inv olved, although less frequently the heart, lung, kidney, genital syste m, and gastrointestinal tract may be affected. The prognosis of the di sease has been improved because of early diagnosis and suitable treatm ent. Local remedies and systemic administration of colchicine, cortico steroids, immunosuppressives, and other agents have been applied. Conc lusion: ED is a widespread vasculitis affecting young people and invol ving concurrently or consecutively nearly all organs and systems. Trea tment results in better prognosis even when vital organs are involved. Copyright (C) 1998 by W.B. Saunders Company.