自身免疫性肝病重叠综合征的现状  

Current progress in research of overlap syndrome of autoimmune liver diseases

作　　者：黄颖秋[1,2] 

机构地区：[1]中国医科大学第五临床学院 [2]本溪钢铁(集团)总医院消化内科,辽宁省本溪市117000

出　　处：《世界华人消化杂志》2014年第3期301-309,共9页World Chinese Journal of Digestology

摘　　要：自身免疫性肝病(autoimmune liver disease,A I L D)包括自身免疫性肝炎(a u t o i m m u n e hepatitis,AIH)、原发性胆汁性肝硬化(primary biliary cirrhosis,PBC)和原发性硬化性胆管炎(primary sclerosing cholangitis,PSC).AILD重叠综合征(overlap syndrome,OS)是指患者同时兼具AIH和PBC或PSC两种疾病的临床表现、生物化学、血清学、组织学以及影像学特征的一种罕见疾病状态.AILD OS主要分为AIH-PBC OS和AIH-PSC OS.前者多见于成人,后者多见于儿童.OS若无治疗,最终可致肝硬化或肝衰竭.高剂量熊去氧胆酸(ursodeoxycholic acid)与免疫抑制剂类固醇和/或硫唑嘌呤(azathioprine)联合使用通常被用于治疗AIH-PBC OS和AIH-PSC OS.目前,肝移植仍是治疗终末期OS患者的唯一有效方法.Autoimmune liver diseases （AILDs） include autoimmune hepatitis （AIH）, primary biliary cir- rhosis （PBC） and primary sclerosing cholangitis （PSC）. Overlap syndrome （OS） of AILDs is a rel- atively infrequent disease that has the combined clinical, biochemical, serological, histological and radiological features of AIH and PBC or PSC, mainly comprising AIH-PBC and AIH-PSC overlap syndromes. The AIH-PBC overlap syn- drome is common in adults, while the AIH-PSC overlap syndrome is predominantly found in children. Overlap syndromes have a progressive course and may finally lead to liver cirrhosis and liver failure without treatment. High-dose ursodeoxycholic acid （UDCA） combined with immunosuppressive therapy with corticoste- folds and/or azathioprine （AZA） is usually used in both AIH-PBC and AIH-PSC overlap syn- dromes. At present, liver transplantation is theonly option with known therapeutic benefit for end-stage patients.

关 键 词：自身免疫性肝病 重叠综合征 自身免疫性肝炎 原发性胆汁性肝硬化 原发性硬化性胆管炎 

分 类 号：R575[医药卫生—消化系统]