【摘要】 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P B C和A I H的双重特征,应对此病充分认识,并探索有效的治疗方案。更多还原

【Abstract】 Objective To study the clinical and pathological characteristics of primary biliary cirrhosis-autoimmune hepatitis(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,such as transpeptidase alkaline phosphsphatase(ALP) and immunoglobulin 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.更多还原