肖巧?肖华?王容?赵川

    【摘要】原发性胆汁性胆管炎(PBC)病因尚不清楚，可能与基因、遗传、环境等因素有关，熊去氧胆酸是该病的一线治疗药物。嗜酸性肉芽肿性血管炎(EGPA)是一种系统性血管炎性疾病，以坏死性血管炎、组织器官嗜酸性粒细胞浸润和血管外肉芽肿形成为主要特征。该文报道1例65岁男性患者以反复四肢皮疹为主要表现，反复肝功能异常，同时存在哮喘样喘息，镜下血尿，肾功能异常及嗜酸性粒细胞增多，最后诊断为PBC合并EGPA。该例提示不明原因嗜酸性粒细胞增多，同时合并皮肤、肺、肝、肾、免疫等多器官损害时，需考虑是否为风湿免疫系统疾病。

    【关键词】原发性胆汁性胆管炎；肝硬化；嗜酸性肉芽肿性血管炎；嗜酸性粒细胞增多症；多器官损害

    Primary biliary cholangitis complicated with eosinophilic granulomatous with polyangiitis： a case report Xiao Qiao， Xiao Hua， Wang Rong， Zhao Chuan. Department of Infectious Diseases， Suining Central Hospital， Suining 629000， China

    Corresponding author， Xiao Qiao， E-mail： 747436105@qq.com

    【Abstract】The etiology of primary biliary cholangitis (PBC) is unclear， which may be associated with genetic， hereditary and environmental factors， etc. Ursodeoxycholic acid (UDCA) is the first-line drug for the treatment of PBC. Eosinophilic granulomatous with polyangiitis (EGPA) is a systemic vascular inflammatory disease characterized by necrotizing vasculitis， infiltration of eosinophils in tissues and organs， and formation of extravascular granulomas. In this report ......