Behçet's Disease.

Abstract

OBJECTIVES: To review the new data on the epidemiology, etiopathogenesis, clinicolaboratory spectrum, prognosis, and treatments of Behçet's disease (BD). 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, and systemic. RESULTS: BD is a multisystem vasculitis with recurrent symptoms. 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 series of patients, men predominate over women. Infectious agents, immune mechanisms, and genetic factors are implicated in the etiopathogenesis of the disease, which remains to be elucidated. The pathology of the lesions consists of widespread vasculitis. Eyes, skin, joints, the oral cavity, blood vessels, and central nervous system are usually involved, although less frequently the heart, lung, kidney, genital system, and gastrointestinal tract may be affected. The prognosis of the disease has been improved because of early diagnosis and suitable treatment. Local remedies and systemic administration of colchicine, corticosteroids, immunosuppressives, and other agents have been applied. CONCLUSION: BD is a widespread vasculitis affecting young people and involving concurrently or consecutively nearly all organs and systems. Treatment results in better prognosis even when vital organs are involved.