自身免疫性肝病相关病理特征

Pathological Features of Autoimmune Liver Diseases

肝脏组织学在自身免疫性肝病(AILD)的诊治中发挥重要作用。AILD主要包括自身免疫性肝炎(AIH)、原发性胆汁性胆管炎(PBC,旧称原发性胆汁性肝硬化)、原发性硬化性胆管炎(PSC)、IgG4相关硬化性胆管炎(IgG4-SC)和肝脏IgG4疾病。AIH组织学特征性表现为界面性肝炎伴淋巴-浆细胞浸润、"玫瑰花环样"肝细胞和淋巴细胞穿入现象;慢性非化脓性破坏性胆管炎伴上皮样肉芽肿形成需考虑PBC;胆管周围"洋葱皮样"纤维化提示PSC;席纹状纤维化伴大量IgG4阳性浆细胞浸润需考虑IgG4-SC。临床医师与病理医师充分联系与沟通有利于提高AILD的诊治水平。

Autoimmune hepatitis（ AIH）,primary biliary cholangitis（ PBC,formerly known as primary biliary cirrhosis）,primary sclerosing cholangitis（ PSC）, IgG4-related sclerosing cholangitis（ IgG4-SC） and IgG4-related hepatopathy are the main categories of autoimmune liver diseases（ AILDs）,and liver biopsy is important for their diagnosis and management. The characteristic histological features of AIH are interface hepatitis with portal lymphoplasmacytic infiltration,hepatic rosette formation and emperipolesis. Chronic nonsuppurative destructive cholangitis and epithelioid granuloma are frequently seen in PBC. PSC is characterized by ‘onion-skin ＇ type periductal fibrosis,while storiform fibrosis with IgG4-positive plasmacytic infiltration usually indicates IgG4-SC. Closer cooperation between clinicians and pathologists may improve the diagnosis and management of AILDs.