原发性胆汁性胆管炎和原发性胆汁性胆管炎-自身免疫性肝炎重叠综合征患者临床与肝组织病理学特点分析*

doi:10.3969/j.issn.1672-5069.2024.05.019

实用肝脏病杂志 ›› 2024, Vol. 27 ›› Issue (5): 721-724.doi: 10.3969/j.issn.1672-5069.2024.05.019

原发性胆汁性胆管炎和原发性胆汁性胆管炎-自身免疫性肝炎重叠综合征患者临床与肝组织病理学特点分析^*

王燕燕, 周桐桐, 卞兆连

226000 江苏省南通市南通大学附属南通第三医院消化内科

收稿日期:2023-10-20 出版日期:2024-09-10 发布日期:2024-09-09
通讯作者: 卞兆连,E-mail:bianzhaolian1998@163.com
作者简介:王燕燕,女,25岁,硕士研究生。E-mail:1293094847@qq.com
基金资助:
^*江苏省“六大高峰人才”科研基金资助项目(编号:YY-177);江苏省“青年医学人才”培养基金资助项目 (编号:QNRC2016400);江苏省“333工程”计划项目(编号:BRA2020196);南通市科技局科研项目(编号:MS22018007);南通市重点医学人才培养计划项目(编号:No.05)

Clinical feature and biochemical response to immunosuppressive therapy in patients with primary biliary cholangitis and PBC-autoimmune hepatitis overlapping syndrome

Wang Yanyan, Zhou Tongtong, Bian Zhaolian

Department of Gastroenterology,ThirdHospital,Affiliated to Nantong University,Nantong 226000,Jiangsu Province,China

Received:2023-10-20 Online:2024-09-10 Published:2024-09-09

摘要/Abstract

摘要： 目的比较分析原发性胆汁性胆管炎(PBC)和PBC-自身免疫性肝炎(AIH)重叠综合征(OS)患者临床特征。方法 2017年5月～2023年2月南通大学附属南通第三医院诊治的PBC患者64例和PBC-AIH OS患者27例。常规进行肝穿刺检查,分别给予熊去氧胆酸(UDCA)或UDCA联合泼尼松(龙)治疗,观察1年。结果 PBC-AIH OS患者血清AST、球蛋白和IgG水平分别为103.5(74.5,213.3)U/L、34.2±7.3g/L和19.1±8.1g/L,显著高于PBC患者【分别为71.0(32.0,119.0)U/L、30.2±6.2g/L和14.7±5.3g/L,P<0.05】;PBC-AIH OS患者肝组织炎症分级、纤维化分期、界面性肝炎、融合坏死和汇管区炎症等方面均显著重于PBC患者(P<0.05);在治疗1年末,64例PBC患者生化学完全应答、部分应答和无应答率分别为56.3%、26.7%和17.2%,而27例PBC-AIH OS患者则分别为44.4%、37.0%和18.5%。结论 PBC和PBC-AIH OS主要发生在中老年女性,两种疾病临床表现和特征性血清自身抗体阳性率无显著性差异,但PBC-AIH OS患者肝组织病理学病变较单纯PBC患者更重,治疗应答相对差,需要认真管理。

关键词: 自身免疫性肝病, 原发性胆汁性胆管炎, 自身免疫性肝炎, 重叠综合征, 临床特征, 治疗应答

Abstract: Objective The clinical feature and biochemical response to immunosuppressive therapy were compared between patients with primary biliary cholangitis (PBC) and PBC-autoimmune hepatitis (AIH)overlap syndrome (OS). Methods 64 patients with PBC and 27 patients with PBC-AIH OS were encountered in our hospital between May 2017 and February 2023, and all undergone liver biopsies. The patients in the two groups were treated by ursodeoxycholic acid (UDCA) or UDCA and prednisone combination, and response were assessed by one year. Results Serum AST, globulin and IgG levels in patients with PBC-AIH OS were 103.5(74.5, 213.3)U/L, 34.2±7.3g/L and 19.1±8.1g/L, all significantly higher than [71.0(32.0, 119.0)U/L,30.2±6.2g/L and 14.7±5.3g/L, respectively, P<0.05] in patients with PBC; histo-patholocially, hepatic activity index, fibrosis staging, interfacial inflammation, fusion necrosis and portal inflammation in patients with PBC-AIH OS were much more severe than in those with PBC(P<0.05); by one year, complete, partial and non-biochemical response were 56.3%, 26.7% and 17.2% in 64 patients with PBC, while they were 44.4%, 37.0% and 18.5% in 27 patients with PBC-AIH OS. Conclusion PBC and PBC-AIH OS predominantly involve in middle-aged and elderly women, and there is little discrepancy in clinical manifestations and characteristic serum autoimmune antibodies between the two groups, while the intrahepatic histo-pathologic damage were obviously severe in patients with PBC-AIH OS, with relatively poor response rate to immunosuppressive therapy, which warrants close surveillance in clinical practice.

Key words: Autoimmune liver disease, Primary biliary cholangitis, Overlap syndrome, Clinical features, Response

王燕燕, 周桐桐, 卞兆连. 原发性胆汁性胆管炎和原发性胆汁性胆管炎-自身免疫性肝炎重叠综合征患者临床与肝组织病理学特点分析^*[J]. 实用肝脏病杂志, 2024, 27(5): 721-724.

Wang Yanyan, Zhou Tongtong, Bian Zhaolian. Clinical feature and biochemical response to immunosuppressive therapy in patients with primary biliary cholangitis and PBC-autoimmune hepatitis overlapping syndrome[J]. Journal of Practical Hepatology, 2024, 27(5): 721-724.

参考文献

[1] Zheng Y, Ran Y, Zhang H, et al. The microbiome in autoimmune liver diseases: metagenomic and metabolomic c. Front Physiol, 2021, 12:715852.
[2] Yan Y L, Xing X, Wang Y, et al. Clinical utility of two-dimensional shear-wave elastography in monitoring disease course in autoimmune hepatitis-primary biliary cholangitis overlap syndrome. World J Gastroenterol, 2022, 28(18): 2021-2033.
[3] Kuiper E M, Zondervan P E, van Buuren H R. Paris criteria are effective in diagnosis of primary biliary cirrhosis and autoimmune hepatitis overlap syndrome. Clin Gastroenterol Hepatol, 2010, 8(6): 530-534.
[4] 张骏飞, 许何明, 宋海燕,等. 自身免疫性肝炎-原发性胆汁性胆管炎重叠综合征:巴黎标准诊断中国患者灵敏度低. 实用肝脏病杂志, 2019, 22(4): 537-540.
[5] 中华医学会肝病学分会.原发性胆汁性肝硬化(又名原发性胆汁性胆管炎)诊断和治疗共识(2015年). 肝脏, 2015, 20(12): 960-968.
[6] 尤红, 段维佳, 李淑香,等. 原发性胆汁性胆管炎的诊断和治疗指南(2021年). 临床肝胆病杂志, 2022, 38(1): 35-41.
[7] Liu F, Pan Z G, Ye J, et al. Primary biliary cirrhosis-autoimmune hepatitis overlap syndrome: simplified criteria may be effective in the diagnosis in Chinese patients. J Dig Dis, 2014, 15(12): 660-668.
[8] 田爱平, 杨永峰. 慢性肝炎病理学分级分期评分系统比较. 临床肝胆病杂志, 2018, 34(11): 2271-2277.
[9] Goodman Z D. Grading and staging systems for inflammation and fibrosis in chronic liver diseases. J Hepatol, 2007, 47(4): 598-607.
[10] Di Tommaso L, Destro A, Seok J Y, et al. The application of markers (HSP70 GPC3 and GS) in liver biopsies is useful for detection of hepatocellular carcinoma. J Hepatol, 2009, 50(4): 746-754.
[11] Chatzipantelis P, Giatromanolaki A. Early histopathologic changes in primary biliary cholangitis: does 'minimal change' primary biliary cholangitis exist? A pathologist's view. Eur J Gastroenterol Hepatol, 2021, 33(12): e7-e12.
[12] Younossi Z M, Bernstein D, Shiffman M L, et al. Diagnosis and management of primary biliary cholangitis. Am J Gastroenterol, 2019, 114(1): 48-63.
[13] Terziroli Beretta-Poccoli B, Mieli-Vergani G, Vergani D, et al. The challenges of primary biliary cholangitis: what is new and what needs to be done. J Autoimmun, 2019, 105(102328.
[14] Medford A, Childs J, Little A, et al. Emerging therapeutic strategies in the fight against primary biliary cholangitis. J Clin Transl Hepatol, 2023, 11(4): 949-957.
[15] Talwalkar J A, Lindor K D. Primary biliary cirrhosis. Lancet, 2003, 362(9377): 53-61.
[16] Lohse A W, Zum Buschenfelde K H, Franz B, et al. Characterization of the overlap syndrome of primary biliary cirrhosis (PBC) and autoimmune hepatitis: evidence for it being a hepatitic form of PBC in genetically susceptible individuals. Hepatology, 1999, 29(4): 1078-184.
[17] Lee B T, Wang Y, Yang A, et al. IgG:IgM ratios of liver plasma cells reveal similar phenotypes of primary biliary cholangitis with and without features of autoimmune hepatitis. Clin Gastroenterol Hepatol, 2021, 19(2): 397-9.
[18] Silveira M G, Lindor K D. Overlap syndromes with autoimmune hepatitis in chronic cholestatic liver diseases. Expert Rev Gastroenterol Hepatol, 2007, 1(2): 329-340.
[19] Degott C, Zzfrani E S, Callard P, et al. Histopathological study of primary biliary cirrhosis and the effect of ursodeoxycholic acid treatment on histology progression. Hepatology, 1999, 29(4): 1007-1012.
[20] Nguyen H H, Shaheen A A, Baeza N, et al. Evaluation of classical and novel autoantibodies for the diagnosis of primary biliary cholangitis-autoimmune hepatitis overlap syndrome (PBC-AIH OS). PLoS One, 2018, 13(3): e0193960.
[21] Sherlock S. Primary biliary cirrhosis, primary sclerosing cholangitis, and autoimmune cholangitis. Clin Liver Dis, 2000, 4(1): 97-113.

原发性胆汁性胆管炎和原发性胆汁性胆管炎-自身免疫性肝炎重叠综合征患者临床与肝组织病理学特点分析^*

Clinical feature and biochemical response to immunosuppressive therapy in patients with primary biliary cholangitis and PBC-autoimmune hepatitis overlapping syndrome

PDF (PC)

可视化

摘要/Abstract

引用本文

使用本文

参考文献

相关文章 15

编辑推荐

Metrics

本文评价

[1]	高怡, 沈小雪, 夏素芹, 张洁, 朱先兰. 自身免疫性肝炎和原发性胆汁性胆管炎患者血清胆汁酸成分变化及其对治疗应答的影响^*[J]. 实用肝脏病杂志, 2024, 27(5): 709-712.
[2]	蒋叶舟, 花霞, 陈国飞, 朱青蓝, 蔡明月. 自身免疫性肝炎患者血清瘦素、外周血调节性T细胞和Th17细胞水平变化^*[J]. 实用肝脏病杂志, 2024, 27(5): 713-716.
[3]	朱家瑞, 葛绍锋, 叶桂云, 黄远茂. 自身免疫性肝炎患者外周血NLR和nCD64指数变化及其临床意义探讨^*[J]. 实用肝脏病杂志, 2024, 27(5): 717-720.
[4]	任艳, 孔明, 徐曼曼, 白丽, 陈煜. 瞬时弹性成像技术和无创诊断模型诊断原发性胆汁性胆管炎患者肝纤维化效能研究^*[J]. 实用肝脏病杂志, 2024, 27(5): 725-728.
[5]	王秀娟, 朱贺, 张茹梦, 常文绘, 刘玉玉. 泼尼松联合硫唑嘌呤治疗自身免疫性肝炎患者不同减量方法的效果研究^*[J]. 实用肝脏病杂志, 2024, 27(5): 729-732.
[6]	王娟, 郑自通, 刘丽华, 何鑫, 姚旭, 彭灿. 接受雷公藤制剂治疗的类风湿性关节炎并发药物性肝损害患者临床特征及预后分析^*[J]. 实用肝脏病杂志, 2024, 27(5): 733-736.
[7]	张丽, 陈雅雯, 盖琳, 邱红, 甄鑫, 常静霞. 乙型肝炎病毒/丙型肝炎病毒共同感染患者临床特征及其对抗病毒治疗应答反应研究^*[J]. 实用肝脏病杂志, 2024, 27(4): 523-526.
[8]	任浩, 熊清芳. 多种无创检测指标预测自身免疫性肝病患者肝硬化效能比较^*[J]. 实用肝脏病杂志, 2024, 27(4): 535-538.
[9]	蒋瑶, 仲伟明, 周俏怡, 邵宁, 吕冰, 孙舒. 吗替麦考酚酯治疗难治性自身免疫性肝炎患者疗效初步研究^*[J]. 实用肝脏病杂志, 2024, 27(4): 539-542.
[10]	潘劲劲, 谢琴秀, 许夕海, 郜玉峰, 李家斌. 药物性肝损伤患者血清抗核抗体阳性临床意义探讨^*[J]. 实用肝脏病杂志, 2024, 27(4): 543-546.
[11]	金伯旬, 张丽, 娜仁图雅, 齐丽艳, 边新渠, 刘海霞, 王鑫, 栗光明. 急性表现与非急性表现的自身免疫性肝炎患者临床和肝组织病理学特征分析^*[J]. 实用肝脏病杂志, 2024, 27(3): 366-369.
[12]	刘恋, 吉飞跃, 陈诺儿. 自身免疫性肝炎患者外周血单个核细胞CD6及其配体激活的白细胞粘附分子水平变化临床价值研究^*[J]. 实用肝脏病杂志, 2024, 27(3): 370-373.
[13]	徐秋萍, 黄彬, 陈敏, 余静. 泼尼松与泼尼松联合硫唑嘌呤治疗自身免疫性肝炎患者外周血MDSCs百分比、肝组织白细胞介素-35和血清CD38水平变化及其临床意义探讨^*[J]. 实用肝脏病杂志, 2024, 27(3): 374-377.
[14]	姜正伟, 周冰清, 汤洁, 蒋蓓莉. 血清抗糖蛋白210抗体阳性的原发性胆汁性胆管炎患者临床特征分析^*[J]. 实用肝脏病杂志, 2024, 27(3): 378-381.
[15]	高萍, 高学松, 张亦瑾, 孙磊, 刘楠, 李洪杰, 刘亚平, 高丽丽, 段雪飞. 8例血色病患者临床与基因突变特征分析^*[J]. 实用肝脏病杂志, 2024, 27(3): 386-389.