自身免疫性肝炎-原发性胆汁性胆管炎重叠综合征患者临床和肝组织病理学特征分析  

Clinical and liver pathological features of patients with autoimmune hepatitis-primary biliary cholangitis overlap syndrome

作　　者：郭银燕 岳枝 盖晓冬 冯艳红 张杰东 Guo Yinyan;Yue Zhi;Gai Xiaodong(Department of Hepatology,Second Hospital,Affiliated to Nanjing,University of Chinese Medicine,Nanjing 210003,Jiangsu Province,China)

机构地区：[1]南京中医药大学附属南京医院(南京市第二医院)肝病科,南京市210003 [2]南京中医药大学附属南京医院(南京市第二医院)病理科,南京市210003

出　　处：《实用肝脏病杂志》2023年第3期372-375,共4页Journal of Practical Hepatology

基　　金：南京市卫生科技发展专项资金一般项目(编号:YKK22127)。

摘　　要：目的 分析自身免疫性肝炎-原发性胆汁性胆管炎重叠综合征(AIH-PBC OS)患者临床和肝组织病理学特征。方法 2018年3月~2022年3月我院诊治的AIH-PBC OS患者42例,接受肝穿刺检查后,给予熊去氧胆酸(UDCA)联合或不联合甲泼尼松龙治疗半年。采用间接免疫荧光法或免疫印迹法等测定抗核抗体(ANA)、抗线粒体抗体(AMA)、抗线粒体抗体Ⅱ型(AMA-M2)、抗核膜糖蛋白210抗体(gp210)和抗可溶性酸性磷酸化核蛋白(sp100)。结果 在42例AIH-PBC OS患者中,女性占85.7%,发病年龄为(53.9±10.6)岁;血清TBIL、Alb和ALP异常率分别为57.1%、60.0%和75.7%;ALT、AST和GGT异常率均超过83.0%,其中GGT异常升高达95.2%,在治疗半年后,血生化指标均大幅下降;血清免疫球蛋白IgG、IgM和IgA异常率分别为69.0%、52.3%和33.3%;血清ANA、AMA、AMA2、gp210和sp100阳性率分别为90.5%、73.5%、72.7%、47.1%和36.7%;常见ANA荧光模型为胞浆颗粒型23例、着丝点型13例和核膜型8例;肝组织均表现为界面性肝炎、肝细胞炎症活动度以轻-中度炎症为主,纤维化程度以轻度为主。结论 AIH-PBC OS多见于女性,在临床和组织病理学上兼有PBC和AIH的双重特点,给予UDCA联合皮质激素治疗有助于改善血生化指标。Objective The purpose of this study was to summarize the clinical and liver pathological features of patients with autoimmune hepatitis-primary biliary cholangitis overlap syndrome(AIH-PBC OS).Methods 42 patients with AIH-PBC OS were encountered in our hospital between March 2018 and March 2022,and all underwent liver biopsies and received ursodeoxycholic acid(UDCA)with or without prednisolone combination therapy for six months.Serum antinuclear antibody(ANA),anti-mitochondrial antibody(AMA),anti-mitochondrial antibody type II(AMA-M2),anti-nuclear membrane glycoprotein 210 antibody(gp210)and anti-soluble acidic phosphorylated nuclear protein(sp100)were detected.Results Among the 42 patients with AIH-PBC OS,85.7%cases were female,and the average age of onset was(53.9±10.6)years;the abnormal rates of serum bilirubin,albumin and ALP levels were 57.1%,60.0%and 75.7%,and the elevated serum ALT,AST and GGT levels were all more than 83.0%,and all but serum albumin,decreased greatly after six month treatment(P<0.05);the increased plasma immunoglobulin IgG,IgM and IgA levels were 69.0%,52.3%and 33.3%;the positive rates of serum ANA,AMA,AMA2,gp210 and SP100 were 90.5%,73.5%,72.7%,47.1%and 36.7%,respectively;the common fluorescence models of ANA were cytoplasmic granules in 23 cases,centromere in 13 cases,and nuclear membrane in 8 cases;the liver histopathological examination showed the interface hepatitis,mainly mild-moderate inflammation,and mild hepatic fibrosis.Conclusion The female patients with AIH-PBC OS is more common,and the disease has the dual features of PBC and AIH.We recommend early administration of UDCA with or without steroid therapy,which might be helpful to improve the prognosis of patients with AIH-PBC OS.

关 键 词：自身免疫性肝炎 原发性胆汁性胆管炎 重叠综合征 临床特征 熊去氧胆酸 甲泼尼松龙 治疗 

分 类 号：R575[医药卫生—消化系统]