原发性胆汁性肝硬化和自身免疫性肝炎重叠综合征患者的临床特征分析

Clinical features of primary biliary cirrhosis-autoimmune hepatitis overlap syndrome.

目的分析原发性胆汁性肝硬化(PBC)和自身免疫性肝炎(AIH)重叠综合征(PBC/AIH重叠综合征)患者的临床表现和肝组织病理学的特点。方法对124例自身免疫性肝病患者的临床资料进行分析,根据国际自身免疫性肝炎小组修订的诊断标准,筛选出19例PBC/AIH重叠综合征患者,分析比较PBC/AIH重叠综合征和单纯PBC、AIH患者的临床表现、肝组织病理学变化。结果124例自身免疫性肝病患者中19例PBC/AIH重叠综合征,单纯PBC55例、AIH49例,原发性硬化性胆管炎(PSC)1例,分别占15.32%、44.35%、39.52%和0.81%。PBC/AIH重叠综合征实验室检查具有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G的明显升高,同时具有PBC的特点,如转肽酶、碱性磷酸酶、免疫球蛋白M的显著增高;自身抗体检测可见抗核抗体、抗平滑肌抗体、抗线粒体抗体、M2抗体阳性;肝组织病理结果显示,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,胆管不同程度病变。结论PBC/AIH重叠综合征并非罕见,它的临床表现、肝组织病理学具有PBC和AIH的双重特征,应对此病充分认识,探索有效的治疗方案。

Objective To study the clinical and pathological characteristics of primary biliary cirrhosis-autoimmune hepatitis overlap syndrome （ PBC/AIH ）. Methods Of 124 patients with PBC,AIH and primary sclerosing cholangitis（PSC） , 19 cases were identified as PBC/AIH syndrome The clinical and pathological characteristics of primary biliary cirrhosis-autoimmune hepatitis overlap syndrome （ PBC/AIH ） , pure PBC, and pure AIH were analyzed. Results 19 patients （15.32%） had PBC/AIH overlap syndrome,55 patients （44.35%）had pure PBC, and 49 patients（39.52% ） had AIH as well as one case （0.81%） had PSC. The overlap syndrome patients had the features of AIH, such as high serum levels of alanine aminotransferase （ ALT ）, aspartate aminotransferase （ AST ）, and immunoglobulin （ IgG ）, and of PBC, such as transpeptidase, alkaline phosphatase （ ALP ）, and immunoglobulin M （ IgM ）, and the features of both AIH and PBC included presence of mitochondrial antibodies （AMA） or ASMA, and anti-M2 ,florid bile duct lesions, and moderate or severe periportal or periseptal lymphocytic piecemeal necrosis. Conelusion The overlap syndrome is not rare,which clinical and pathological characteristics are overlapped with both PBC and AIH, so it is necessary to fully understand the disease.