中晚期自身免疫性肝炎-原发性胆汁性肝硬化重叠综合征的临床病理研究

目的探讨中晚期自身免疫性肝炎-原发性胆汁性肝硬化（AIH-PBC）重叠综合征的临床病理特征及治疗直答。方法对具有肝穿刺标本的11例PBC-AIH重叠综合征和13例PBC（Seheuer分期3、4期）患者进行比较，重点分析AIH-PBC重叠综合征的临床、病理特点及治疗应答。结果两组患者的性别、年龄、病程、症状无显著差异；AIH-PBC重叠综合征患者的丙氨酸氨基转移酶、天冬氨酸氨基转移酶、γ-球蛋白、免疫球蛋白IgG以及抗核抗体或抗平滑肌抗体阳性率明显高于PBC（P〈0．05）。肝组织学见汇管区与肝腺泡内以单个核细胞为主的较多炎细胞浸润，其中易见浆细胞的聚积性浸润。可见不同时期小胆管损伤或毛细胆管反应性增生并侵蚀肝界板；重叠综合征患者经熊去氧胆酸治疗可使肝功能改善，与PBC患者无明显差异。结论中晚期AIH-PBC重叠综合征临床、血清学及组织病理学表现出AIH和PBC双重特征，UDCA治疗有助于血生化指标的改善。

Clinical and pathological studies on the autoimmune hepatitis and primary biliary cirrhosis overlap syndrome :an analysis of 11 cases

Objective To expiore the clinical and pathological features and responses to therapy of autoimmune hepatitis and primary biliary cirrhosis overlap syndrome. Methods 11 cases with AIH-PBC overlap syndrome and 13 cases with PBC （Scheuer stage 3 and 4） ,were included,and the emphases was laid upon the clinical manifestations,pathological features and responses to therapy. Results No significant differences in sex,age and course of diseases were found between the 2 groups. In AIH-PBC group, the levels of alanine aminotransaminase,aspartic aminotransaminase, γ-globulin and IgG and the presence of positive antinuclear antibody or antismooth muscle antibody were markedly higher than those in PBC group （P〈0.05）. The pathological findings in the AIH-PBC group included moderate to severe interface hepatitis with plasma cell predominated mixed inflammatory cells infiltration in hepatic and portal tracts,as well as the damage and obvious reactive proliferation of small bile ducts. The liver function tests improved by UDCA treatment in AIH-PBC patients. Conclusion A combined features of both AIH and PBC exists in overlap syndrome,differrent from AIH or PBC alone. The administration of UDCA may benefit.