【摘要】 目的 以自身免疫性肝炎(autoimmune hepatitis, AIH)、原发性胆汁性胆管炎(primary biliary cholangitis, PBC)、AIH-PBC重叠综合征(overlap syndrome, OS)和药物诱发性自身免疫样肝炎(drug-induced autoimmune-like hepatitis, DI-AILH)为研究对象，结合临床特征、组织病理、免疫组织化学染色和免疫荧光染色展开多方面研究，以探寻有助于鉴别诊断它们的临床病理特征。方法 对各组患者的肝穿刺标本进行HE、免疫组化和免疫荧光染色，结合判读结果量化分析AIH、PBC和OS之间及AIH和DI-AILH之间的组织学、免疫组织化学和免疫荧光差异；并针对DI-AILH和AIH之间显著性差异因子建立Logistic回归模型。结果 组织学评估中，AIH出现显著的界面性肝炎、Rosette花环形成、界板处成簇的浆细胞浸润；PBC出现汇管区浆细胞浸润、明显的淋巴细胞性小叶间胆管炎；OS出现更高的汇管区和肝小叶内炎症分级。免疫组化和免疫荧光半定量评分显示，AIH和PBC在汇管区和界板处阳性细胞有显著性差异、AIH出现IgG小叶内和汇管区沉积、PBC出现IgA沿小叶间胆管线状沉积。DI-AILH和AIH浆细胞、中性粒细胞浸润和界板处炎症差异明显，Logistic回归模型曲线下面积>0.80。结论 尽管在临床病理特点方面存在重叠，AIH、PBC和AIH-PBC OS仍存在有助于各自诊断的组织学、免疫组化及荧光特点，根据肝组织损伤模型，病理医师也可以给出关于AIH和DI-AILH较为准确的诊断。更多还原

【Abstract】 Objective Autoimmune hepatitis(AIH), primary biliary cholangitis(PBC), AIH-PBC overlap syndrome(OS) and drug-induced autoimmune-like hepatitis(DI-AILH) were studied by combining clinical features, histopathology, immunohistochemical staining and immunofluorescence staining. To explore the clinicopathological features that are helpful for their differential diagnosis. Methods The histological, immunohistochemical and immunofluorescence differences among AIH, PBC and OS. AIH and DI-AILH were quantitatively analyzed. Logistic regression model was established according to the significant difference factors between DI-AILH and AIH. Results As for histological evaluation, AIH showed significant interface hepatitis, Rosette formation, and clustered plasma cell infiltration at the boundary plate. There were plasma cell infiltration and obvious lymphocytic interlobular cholangitis in the portal area of PBC. OS showed higher grades of portal and intralobular inflammation. Immunohistochemical and immunofluorescence semi-quantitative scores showed significant differences in the positive cells in the portal area and the boundary plate between AIH and PBC, intralobular and portal deposition of IgG in AIH, linear deposition of IgA along interlobular bile ducts in PBC. There were significant differences in plasma cells, neutrophil infiltration and lamina inflammation between DI-AILH and AIH, and the area under the curve of the Logistic regression model was greater than 0.80.Conclusion Despite the overlap in clinicopathological features, AIH, PBC and AIH-PBC OS still have their own histological, immunohistochemical and fluorescence characteristics that are helpful for diagnosis. According to the model of liver tissue injury, pathologists can also provide accurate diagnosis of AIH and DI-AILH.更多还原