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Behçet Disease: Clinical Features and Management in a Brazilian Tertiary Hospital

Background Behçet disease (BD) is prevalent in Central and East Asia and in the Eastern Mediterranean area where most studies have been performed. Few studies have evaluated patients with BD in Brazil. Objectives The objective of the study was to describe clinical manifestations of BD and their therapy in a single center in Brazil. Methods Sixty patients who met the International Study Group Criteria for BD and were under follow-up at the Vasculitis Unit of the Universidade Federal de São Paulo were evaluated in a retrospective observational study. Results Mean age at study was 40.0 (SD, 10.7) years, and the female-male ratio was 1.2:1.0. The frequency of disease manifestations was as follows: oral ulcers, 100%; genital ulcers, 93.3%; ocular manifestations, 63.3%; arthritis, 46.7%; cutaneous lesions, 71.7%; positive pathergy test, 22.7%; neurologic involvement, 28.3%; thrombosis, 13.3%; and gastrointestinal involvement, 3.3%. Arthritis and erythema nodosum were more prevalent among women, whereas papulopustular lesions were more common in men. The frequency of each treatment modality was as follows: colchicine, 78.3%; thalidomide, 26.7%; colchicine and penicillin, 21.7%; dapsone, 8.3%; and pentoxyphyline, 8.3%. These treatments were mainly used for mucocutaneous manifestations. Immunosuppressive drugs were prescribed for 70% of the patients, including azathioprine (35.0%), cyclophosphamide (28.3%), cyclosporin A (21.7%), methotrexate (18.3%), and chlorambucil (6.7%). Infliximab was used in 5.0% of refractory patients. No differences were observed between sexes related to severe manifestations of BD. Conclusions Although reported elsewhere, Brazilian men with BD did not have a worse prognosis. Women had a higher frequency of arthritis manifestations.