原发性胆汁性胆管炎伴嗜酸性肉芽肿性血管炎一例

Primary biliary cholangitis complicated with eosinophilic granulomatous with polyangiitis:a case report

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。

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,a 65-year-old male patient presented with recurrent rashes on four limbs,recurrent abnormal liver function,asthma-like wheezing,microscopic hematuria,abnormal renal function,and eosinophilia.He was finally diagnosed with PBC complicated with EGPA.This case suggests that the possibility of rheumatic immune system disease should be considered for patients with unexplained eosinophilia complicated with multiple organ damage,such as skin,lung,liver,kidney,and immune system.