临床肝胆病杂志
臨床肝膽病雜誌
림상간담병잡지
CHINESE JOURNAL OF CLINICAL HEPATOLOGY
2014年
5期
385-389
,共5页
自身免疫疾病%肝疾病%病理学,临床%组织学%述评
自身免疫疾病%肝疾病%病理學,臨床%組織學%述評
자신면역질병%간질병%병이학,림상%조직학%술평
autoimmune diseases%liver disease%pathology clinical%histology%editorial
肝脏组织学在自身免疫性肝病的诊断中起着重要作用，尤其是临床表现非特异、自身抗体阴性的患者。介绍了几种自身免疫性肝病在组织学上的特征性表现。自身免疫性肝炎（AIH）的特征性表现包括界面性肝炎伴淋巴-浆细胞浸润、玫瑰花环及穿入现象，而慢性非化脓性胆管炎、上皮样肉芽肿常提示原发性胆汁性肝硬化（PBC）。胆管周围洋葱皮样纤维化是原发性硬化性胆管炎（PSC）特征性表现，席纹状纤维化伴IgG4阳性浆细胞浸润常须考虑为IgG4相关硬化性胆管炎。最后指出，在日常工作中，临床医生和病理医生加强沟通有利于提高自身免疫性肝病的诊治。
肝髒組織學在自身免疫性肝病的診斷中起著重要作用，尤其是臨床錶現非特異、自身抗體陰性的患者。介紹瞭幾種自身免疫性肝病在組織學上的特徵性錶現。自身免疫性肝炎（AIH）的特徵性錶現包括界麵性肝炎伴淋巴-漿細胞浸潤、玫瑰花環及穿入現象，而慢性非化膿性膽管炎、上皮樣肉芽腫常提示原髮性膽汁性肝硬化（PBC）。膽管週圍洋蔥皮樣纖維化是原髮性硬化性膽管炎（PSC）特徵性錶現，席紋狀纖維化伴IgG4暘性漿細胞浸潤常鬚攷慮為IgG4相關硬化性膽管炎。最後指齣，在日常工作中，臨床醫生和病理醫生加彊溝通有利于提高自身免疫性肝病的診治。
간장조직학재자신면역성간병적진단중기착중요작용，우기시림상표현비특이、자신항체음성적환자。개소료궤충자신면역성간병재조직학상적특정성표현。자신면역성간염（AIH）적특정성표현포괄계면성간염반림파-장세포침윤、매괴화배급천입현상，이만성비화농성담관염、상피양육아종상제시원발성담즙성간경화（PBC）。담관주위양총피양섬유화시원발성경화성담관염（PSC）특정성표현，석문상섬유화반IgG4양성장세포침윤상수고필위IgG4상관경화성담관염。최후지출，재일상공작중，림상의생화병리의생가강구통유리우제고자신면역성간병적진치。
Liver biopsy plays an important role in the diagnosis of autoimmune liver diseases,particularly in patients without specific clinical manifestations or negative for autoantibodies.The histological features of several autoimmune liver diseases are reviewed.The characteristic features of autoimmune hepatitis in histology include interface hepatitis with lymphoplasmacytic infiltration,hepatic rosette formation,and emperipolesis,while chronic nonsuppurative destructive cholangitis and epithelioid granuloma are frequently found in primary biliary cirrho-sis.Primary sclerosing cholangitis is characterized by onion skin-like periductal fibrosis,while storiform fibrosis with IgG4-positive plas-ma cell infiltration often indicates IgG4 -related sclerosing cholangitis.Enhanced communication between clinicians and pathologists can help improve the diagnosis and treatment of autoimmune liver diseoses.
