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分析

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

目的：总结原发性胆汁肝硬化-自身免疫性肝炎重叠综合征患者的临床及病理组织学特点 方法：对10例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及病理资料进行回顾性分析 结果：本组患者均有肝功能酶谱的不同程度升高，血清 IgG 和 IgM 升高，10例患者血清抗线粒体抗体(antimitochondrial antibody,AMA)及 AMA-M2阳性，其中9例患者抗核抗体(antinuclear a(?)tiboby，ANA)阳性，1例肝肾微粒体抗体(liver kid-ney microsome，IKM)阳性 10例患者肝穿刺活检示均有小胆管损害，肝细胞炎症活动度从中度到重度，纤维化程度从 S1到 S3 结论：自身免疫性肝病重叠综合征患者中女性多见，在临床及病理组织学(?)兼有原发性胆汁性肝硬化和自身免疫性肝炎的双重特点，临床工作中需结合临床、生物化学。

Objective:To explore clinical and histopathological characteristics of primary biliary cirrho-sis-autoimmune hepatitis overlap syndrome.Methods:Clinical data and pathological findings of 10 pa-tients were reviewed.Results:Serum glutamine transpeptidase,alkaline phosphatase levels,alaninetransaminase,aspartate transaminase,serum IgG and IgM were elevated in all the patients.They were allpositive for anti-mitochondrial antibody and AMA-M2.Nine patients were positive for anti-nuclear anti-body and one patient was positive for anti liver-kidney microsome antibody.Liver biopsies in these pa-tients revealed:ten patients had bile duct lesion,hepatitis activities ranged from moderate to severe,andfibrosis ranged from S1 to S3.Conclusion:PBC-AIH overlap syndrome is mostly found in middle-agedwomen.It has the clinical and histopathological characteristics of both PBC and AIH.Accurate andprompt diagnosis of overlap syndrome patients should be based on the clinical presentation,biochemicaland immune indexes,and hepalic pathological changes.