白塞病42例临床分析

目的：探讨白塞病（BD）的临床特点。方法：回顺性分析42例BD患者的临床及实验室资料。结果：BD好发于青壮年，多慢性起病，可累及多个器官系统，各受累部位的频率依次为：口腔42例（100％），生殖器29例（69．0％），皮肤28例（66．7％），关节18例（42．9％），眼18例（42．9％），神经系统6例（14．3％），消化道4例（9．5％），大血管3例（7．1％），呼吸系统2例（4．8％），肾脏1例（2．4％），心脏1例（2．4％）。针刺反应阳性32例（76．2％）。皮肤组织活检12例，病理改变均符合皮肤血管炎。随访发现5例死亡个案中2例为BD肠病，1例为BD肺病，1例为BD脑病。结论：BD是一种以血管炎为基础的可累积全身多器官系统的免疫性疾病，重要脏器受累预后不良。皮质类固醇激素或联合应用免疫抑制剂有较好的临床疗效，但亦带来一定的副作用。

A Clinical Study on 42 Patients with Behcet''''s Disease

Objective:To investigate on the clinical characteristics and laboratory findings in patiets with Behcet's disease(BD).Methods: Retrospectively evaluated the clinical and laboratory data of 42 patients with BD.Results: Most cases in this group were middle-aged individuals with chronic onset.The prevalence of mucocutaneous lesions and systemic manifestations were as following:100% oral ulcer,69% genital ulcer,66.7% cutaneous lesions,42.9%articular involvement and 42.9% ocular involvement,14.3% neurologic involvement,9.5% gastrointestinal involvement,7.1% vascular involvement,4.8% pulmonary involvement,2.4% renal involvement and 2.4% cardiovascular involvement.76.2% of those cases were showed positive pathergy test.Histopathological finding revealed vasculitis.The follow-up showed that 2 patients died of gastrointestinal involvement,while another 2 cases died of pulmonary involvement and neurologic invovement respectively.Conclusion:Behcet's disease is a disorder involved multisystem in which vasculitis is underlying histopathologic changes.Patients with vital organ involvements had a worse prognosis.Steroids combined with immunosuppressive agent were showed the good response,however some side-effects have to be monitored.