原发性胆汁性肝硬化／自身免疫性肝炎重叠综合征患者的临床和病理学研究

目的分析原发性胆汁性肝硬化（PBC）、自身免疫性肝炎（AIH）和PBC／AIH重叠综合征患者的临床和病理学特点。方法对105例自身免疫性肝病患者的临床资料进行分析，比较PBC／AIH重叠综合征和单纯PBC或AIH患者的临床表现和肝组织病理学变化。结果在105例患者中，包括11例PBC／AIH重叠综合征、60例PBC、33例AIH和1例原发性硬化性胆管炎（PSC）。PBC／AIH重叠综合征与PBC或AIH患者的性别、年龄、症状和并发症无明显差异（P〉0．05），其实验室检查具有AIH的特点，如血清丙氨酸氨基转移酶、天冬氨酸氨基转移酶、免疫球蛋白IgG的明显升高，同时具有PBC的特点，如GGT、ALP、免疫球蛋白IgM的显著增高，但与PBC或AIH相比，无统计学差异（P〉0．05）；自身抗体检测可见抗核抗体、抗线粒体M2抗体和抗核心蛋白gP210抗体阳性，后两项抗体检出率明显高于AIH患者（P〈0．01）；肝组织病理学检查结果显示，PBC／AIH重叠综合征兼有PBC和AIH的特点，如界面炎和碎屑样坏死，汇管区浆细胞浸润，胆管不同程度的病变等。结论PBC／AIH重叠综合征的临床表现和肝组织病理学具有PBC和AIH的双重特征，应对此病充分认识，并探索有效的治疗方案。

Clinical and pathological feature of primary biliary cirrhosis-autoimmune hepatitis overlap syndrome

Objective To study the clinical and pathological characteristics of primary biliary cirrhosis-autoimmune bepatitis（PBC/AIH） overlap syndrome. Methods Of 105 patients with autoimmune liver disease, 11 cases were diagnosed as PBC/AIH overlap syndrome by using the international criteria. The clinical and pathological characteristics of 11 PBC/ AIH overlap syndrome,60 PBC and 33 AIH were analyzed. Results No significant differences in gender,age,clinical symptoms and complications were found between the three groups （P〉0.05）. The overlap syndrome patients had the features of AIH,such as high serum levels of alanine aminotransferase,aspartate aminotransferase,immunoglobulin G（IgG）,and the features of PBC,sueh as transpeptidase alkaline phosphsphatase （ALP） and immunoglobulim M（IgM）. ANA,AMA-M2 and anti-gp210 were found in PBC/AIH and the positive rates of AMA-M2 and anti-gp210 were remarkably higher than in AIH（P〈0.05）. The pathological findings in the overlap syndrome showed combined features of interface hepatitis,piecemeal necrosis,and characteristics of PBC. Conclusion PBC/AIH overlap syndrome is not rare,which has clinical and pathological characteristics of both PBC and AIH.