Department of Medicine, Universidade Federal de São Paulo, São Paulo, Brazil.
J Clin Rheumatol. 2011 Dec;17(8):416-20. doi: 10.1097/RHU.0b013e31823a46ed.
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.
The objective of the study was to describe clinical manifestations of BD and their therapy in a single center in Brazil.
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.
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.
Although reported elsewhere, Brazilian men with BD did not have a worse prognosis. Women had a higher frequency of arthritis manifestations.
白塞病（BD）在中亚和东地中海地区流行，这些地区进行了大多数研究。在巴西，很少有研究评估白塞病患者。
本研究的目的是描述巴西单一中心的白塞病患者的临床表现和治疗情况。
在一项回顾性观察研究中，评估了在巴西圣保罗联邦大学血管炎科接受随访的 60 名符合国际研究组白塞病标准的患者。
研究时的平均年龄为 40.0（SD，10.7）岁，男女比例为 1.2:1.0。疾病表现的频率如下：口腔溃疡，100%；生殖器溃疡，93.3%；眼部表现，63.3%；关节炎，46.7%；皮肤损伤，71.7%；阳性帕特格试验，22.7%；神经系统受累，28.3%；血栓形成，13.3%；胃肠道受累，3.3%。关节炎和结节性红斑在女性中更为常见，而脓疱性皮损在男性中更为常见。每种治疗方式的频率如下：秋水仙碱，78.3%；沙利度胺，26.7%；秋水仙碱和青霉素，21.7%；氨苯砜，8.3%；和己酮可可碱，8.3%。这些治疗主要用于黏膜皮肤表现。70%的患者使用了免疫抑制剂，包括硫唑嘌呤（35.0%）、环磷酰胺（28.3%）、环孢素 A（21.7%）、甲氨蝶呤（18.3%）和苯丁酸氮芥（6.7%）。5.0%的难治性患者使用了英夫利昔单抗。在与白塞病严重表现相关的性别方面，没有观察到差异。
尽管在其他地方有报道，但巴西男性白塞病患者的预后并不差。女性关节炎表现的频率更高。
