自身免疫性肝炎-原发性胆汁性肝硬化重叠综合征12例临床分析

Clinical Analysis of 12 Patients with Autoimmune Hepatitis-Primary Biliary Cirrhosis Overlap Syndrome

目的初步探讨自身免疫性肝炎和原发性胆汁性肝硬化重叠综合征(AIH-PBC OLS)的临床特点。方法回顾性总结北京军区总医院近10年收治12例AIH-PBC临床表现、血清生化指标和免疫指标以及病理及治疗。结果 AIH-PBC OLS临床表现缺乏特异性,主要有乏力、低热、黄疸、皮肤瘙痒等;肝脏酶学指标ALT、AST、ALP、GGT、IgM、IgG均明显升高,肝穿刺病理也显示肝细胞坏死、汇管区炎细胞浸润,同时可见小胆管损伤。治疗上倾向以UDCA与皮质激素或免疫抑制剂联合用药。结论 AIH-PBC OLS不完全等同于典型的AIH或者PBC,而更像是两种疾病的同时叠加,临床应严密监控,随时调整治疗方案。

Objective To explore clinical features of autoimmune hepatitis-primary biliary cirrhosis overlap syndrome（ AIH-PBC OLS）. Methods Clinical manifestations,serous biochemical indicators,immune indicators,clinical data of pathological changes and treatments of 12 patients with AIH-PBC OLS in last 10 years were retrospectively summarized. Results Clinical manifestations of AIH-PBC OLS were non-specific in terms of fatigue,low-grade fever,jaundice and pruritus,etc. Hepatic enzymology indexes of ALT,AST,ALP,GGT,IgM and IgG were significantly elevated,and pathological tests of liver biopsy showed hepatocellular damage such as piecemeal necrosis and lymphocytic infiltrates, and simultaneous biliary duct injuries. UDCA combined with corticosteroids or immunosuppressive agents was recommended in the treatment for AIH- PBC OLS. Conclusion The AIH-PBC OLS has relatively specific features,and the manifestations are not similar to those of typical AIH or PBC,but more of manifestations of the two coexisting diseases. So it is necessary to monitor the clinical status closely and adjust treatment protocols.