Objective To investigate the clinical and pathological features of autoimmune hepatitis (AIH) -primary biliary cholangitis (PBC) overlap syndrome (AIH-PBC OS) versus AIH, since it is still difficult to diagnose AIH-PBC OS in clinical practice, and to enhance the knowledge of AIH-PBC OS and improve its early diagnostic rate.Methods A prospective study was performed for the clinical data, biochemical and immunological markers, autoantibody, and liver pathology of 50 patients with AIH-PBC OS confirmed by liver biopsy who visited West China Hospital, Sichuan University, from January 20, 2015 to November 28, 2016 and 57 patients with AIH alone, in order to compare the features of AIH-PBC OS and AIH.Continuous data such as laboratory markers were expressed as median M (inter-quartile range) and the nonparametric rank sum test was used for comparison of these data between two groups;categorical data including clinical features、histopathological manifestations were expressed as rates and the chi-square test was used for comparison of these data between two groups.Results The AIH-PBC OS group had a significantly lower age of onset than the AIH group (Z=-1.975, P=0.048) , while there was no significant difference in male/female sex ratio between the two groups.The AIH-PBC OS group had a significantly higher proportion of patients with pruritus than the AIH group (22.0% vs 7.0%, χ2=4.960, P=0.026) .Compared with the AIH group, the AIH-PBC OS group had significantly higher levels of alkaline phosphatase (ALP) , gamma-glutamyl transpeptidase (GGT) , and immunoglobulin M (IgM) (Z=-6.250, -4.789, and-5.407, all P<0.001) , a significantly higher positive rate of antimitochondrial antibody (AMA) (χ2=26.811, P<0.001) , and significantly lower serum levels of alanine aminotransferase (ALT) , aspartate aminotransferase, and immunoglobulin G (IgG) (Z=-2.916, -3.381, and-2.285, P=0.004, 0.001, and 0.022) .The AIH-PBC OS group had significantly higher incidence rates of bile duct injury, bile duct loss, granulomatous disease, and cholestasis than the AIH group (χ2=25.612, 15.953, 6.490, and 10.042, P<0.001, <0.001, 0.001, and 0.002) .Conclusion Although AIH-PBC OS has the features of both AIH and PBC, it is not a simple overlap between AIH and PBC.Pruritus, significant increases in the levels of ALP, GGT, and IgM, positive AMA, and inflammatory bile duct disease and cholestasis may have implications for the diagnos of AIH-PBC OS.Since AIH-PBC OS may have milder liver inflammatory injury and autoimmune abnormalities than AIH, the levels of ALT and IgG in Paris criteria can be broadened during the diagnosis of AIH-PBC OS, in order to prevent missed diagnosis and avoid affecting the selection of treatment regimen and patients' prognosis.
[1]CHAZOUILLERES O, WENDUM D, SERFATY L, et al.Primary biliary cirrhosis-autoimmune hepatitis overlap syndrome:clinical features and response to therapy[J].Hepatology, 1998, 28 (2) :296.
|
[2]WOODWARD, NEUBERGER J.Autoimmune overlap syndromes[J].Hepatology, 2001, 33 (4) :994.
|
[3]SCHRAMM C, LOHSE AW.Overlap syndromes of cholestatic liver diseases and auto-immune hepatitis[J].Clin Rev Allergy Immunol, 2005, 28 (2) :105-114.
|
[4]European Association for the Study of the Liver.EASL clinical practice guidelines:management of cholestatic liver diseases[J].JHepatol, 2009, 51 (2) :237-267.
|
[5]BUNCHORNTAVAKUL C, REDDY KR.Diagnosis and management of overlap syndromes[J].Clin Liver Dis, 2015, 19 (1) :81-97.
|
[6]KRAWITT EL.Autoimmune hepatitis[J].N Eng I J Med, 2006, 354 (1) :54-66.
|
[7]CHEN CW, CHENG J, DOU XG, et al.Consensus on the diagnosis and management of primary biliary cirrhosis (cholangitis) (2015) [J].J Clin Hepatol, 2015, 20 (12) :960-968. (in Chinese) 陈成伟, 成军, 窦晓光, 等.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) [J].临床肝胆病杂志, 2015, 20 (12) :960-968.
|
[8]BOBERG KM, CHAPMAN RW, HIRSCHFIELD GM, et al.International Autoimmune Hepatitis Group.Overlap syndromes:the international autoimmune hepatitis group (IAIHG) position statement on a controversial issue[J].J Hepatol, 2011, 54 (2) :374-385.
|
[9]KUIPER EM, ZONDERVAN PE, van BUUREN HR.Paris criteria are effective in diagnosis of primary biliary cirrhosis and autoimmune hepatitis overlap syndrome[J].Clin Gastroenterol Hepatol, 2010, 8 (6) :530-534.
|
[10]POUPON R, CHAZOUILLERES O, CORPECHOT C, et al.Development of autoimmune hepatitis in patients with typical primary biliary cirrhosis[J].Hepatology, 2006, 44 (1) :85.
|
[11]SASAKI R, AKIYAMA M, TAKESHITA S, et al.A case of PBC-AIH overlap syndrome who developed AIH 8 years after diagnosis of typical PBC[J].Kanzo, 2009, 50 (50) :445-450.
|
[12]MANNS MP, CZAJA A J, GORHAM JD, et al.AASLD Practice Guidelines.Diagnosis and management of autoimmune hepatitis[J].Hepatology, 2010, 51 (6) :2193-2213.
|
[13]HENNES EM, ZENIYA M, CZAJA AJ, et al.Simplified criteria for the diagnosis of autoimmune hepatitis[J].Hepatology, 2008, 48 (1) :169.
|
[14]CZAJKOWSKI MA, KINGHAM J GC.636 Prevalence and characteristics of overlap syndromes in autoimmune liver disease (AILD) , a twenty-year cohort from a defined community in South Wales[J].J Hepatol, 2006, 44 (Suppl 2) :s236.
|
[15]PARK Y, CHO Y, CHO EJ, et al.Retrospective analysis of autoimmune hepatitis-primary biliary cirrhosis overlap syndrome in Korea:characteristics, treatments, and outcomes[J].Clin Mol Hepatol, 2015, 21 (2) :150-157.
|
[16]Chinese Society of Hepatology, Chinese Medical Association;Chinese Society of Gastroenterology, Chinese Medical Association;Chinese Society of Infectious Diseases, Chinese Medical Association.Chinese consensus on the diagnosis and management of autoimmune hepatitis (2015) [J].J Clin Hepatol, 2016, 32 (1) :9-22. (in Chinese) 中华医学会肝病学分会, 中华医学会消化病学分会, 中华医学会感染病学分会.自身免疫性肝炎诊断和治疗共识 (2015) [J].临床肝胆病杂志, 2016, 32 (1) :9-22.
|
[17]VILLAMIL AG, WELZ G, BANDI JC, et al.556 Overlap syndrome PBC/AIH:prevalence, long term evolution and prognostic factors[J].J Hepatol, 2004, 40 (4) :163.
|
[18]OWENDUM C.Long term outcome and response to therapy of primary biliary cirrhosis-autoimmune hepatitis overlap syndrome[J].J Hepatol, 2006, 44 (2) :400-406.
|
[19]DEGOTT C, ZAFRANI ES, CALLARD P, et al.Histopathological study of primary biliary cirrhosis and the effect of ursodeoxycholic acid treatment on histology progression[J].Hepatology, 1999, 29 (4) :1007-1012.
|
[20]LINDOR KD, GERSHWIN ME, POUPON R, et al.Primary biliary cirrhosis[J].Hepatology, 2009, 50 (1) :291-308.
|
[21]HEURGUE A, VITRY F, DIEBOLD MD, et al.Overlap syndrome of primary biliary cirrhosis and autoimmune hepatitis:a retrospective study of 115 cases of autoimmune liver disease[J].Gastroenterol Clin Biol, 2007, 31 (1) :17-25.
|
[22]MIAO Q, MA X.Significance of clinical pathology in diagnosis and treatment of autoimmune liver diseases[J].J Clin Hepatol, 2014, 30 (5) :385-389. (in Chinese) 苗琪, 马雄.临床病理学在自身免疫性肝病诊治中的重要性[J].临床肝胆病杂志, 2014, 30 (5) :385-389.
|
1. | 李容容,苟悦,刘宁宁,陈日润,孙鑫,刘成海. 基于临床数据挖掘的中药对抗肿瘤药物相关肝损伤的影响及用药规律探讨. 时珍国医国药. 2024(01): 243-247 . ![]() | |
2. | 张倩茹,卢颖,张婉婷,杨艳. 某院急性白血病患者药源性肝损伤临床治疗的回顾性分析. 遵义医科大学学报. 2024(04): 401-407 . ![]() | |
3. | 中华医学会,中华医学会杂志社,中华医学会肝病分会药物性肝病学组,中华医学会全科医学分会,中华医学会《中华全科医师杂志》编辑委员会,中国医药生物技术协会药物性肝损伤防治技术专业委员会,中国初级卫生保健基金会药物肝脏安全性专业委员会,中国药物性肝损伤基层诊疗与管理指南制定专家组. 中国药物性肝损伤基层诊疗与管理指南(2024年). 中华全科医师杂志. 2024(08): 813-830 . ![]() | |
4. | 郑晖,孙蓉. 药物联合应用对中草药相关肝损伤的影响. 临床肝胆病杂志. 2024(08): 1519-1524 . ![]() | |
5. | 李容容,李盟,苟悦,罗琼,吕桦,孙鑫,刘成海. 113例抗肿瘤药物相关肝损伤的临床特点及危险因素分析. 中国药物警戒. 2023(05): 505-510 . ![]() | |
6. | 中国医药生物技术协会药物性肝损伤防治技术专业委员会,中华医学会肝病学分会药物性肝病学组. 中国药物性肝损伤诊治指南(2023年版). 中华肝脏病杂志. 2023(04): 355-384 . ![]() | |
7. | 程诗思,杨成明,曾安津. 荆州地区结核病专科医院抗结核药物不良反应分析. 临床合理用药. 2023(22): 147-150 . ![]() | |
8. | 江程,李春晓,杨玉晴,郭静. 热毒宁注射液上市后临床安全性文献研究. 中国药事. 2023(11): 1252-1265 . ![]() | |
9. | 王双双,熊清芳,胡一帆,陈妙洋,杨永峰. 药物性肝损伤的临床与病理特点分析. 肝脏. 2023(11): 1280-1284 . ![]() | |
10. | 孟尧,张萌萌,郭甜甜,赵新颜. 《中国药物性肝损伤诊治指南(2023年版)》更新要点解读. 中国肝脏病杂志(电子版). 2023(04): 1-5 . ![]() | |
11. | 马世武,刘成海,刘晓琰,苏明华,李东良,李异玲,陈公英,陈军,陈金军,茅益民,赵景民,郭晓燕,唐洁婷,诸葛宇征,谢青,谢雯,赖荣陶,蔡大川,蔡庆贤. 中国药物性肝损伤诊治指南(2023年版). 胃肠病学. 2023(07): 397-431 . ![]() | |
12. | 孙晓楠,吕萌,程国亭. 药物性肝损伤139例回顾性分析. 中南医学科学杂志. 2022(03): 391-394 . ![]() | |
13. | 李娜. 肺癌患者化疗后药物性肝损伤的临床特点. 中国医药指南. 2022(16): 22-25 . ![]() | |
14. | 劳明珠. 异甘草酸镁治疗抗肿瘤药物引起的急性药物性肝损伤临床效果和安全性. 临床合理用药杂志. 2022(17): 84-87 . ![]() | |
15. | 马万龙,焦运,张旭,丁向春,张平,欧阳花,王梦甜,张乐,徐灵博,杨安宁,姜怡邓. 血清IL-6在人工肝治疗药物性肝损伤预后评估中的价值. 宁夏医科大学学报. 2022(06): 622-629 . ![]() | |
16. | 沈婷婷,李光耀,罗琼,李盟,孙鑫,陶艳艳,周祖山,刘成海. 类风湿性关节炎患者应用雷公藤制剂及合并用药所致药物性肝损伤的临床特征分析. 临床肝胆病杂志. 2022(09): 2067-2072 . ![]() | |
17. | 马世武,刘成海,刘晓琰,苏明华,李东良,李异玲,陈公英,陈军,陈金军,茅益民,赵景民,郭晓燕,唐洁婷,诸葛宇征,谢青,谢雯,赖荣陶,蔡大川,蔡庆贤. 中国药物性肝损伤诊治指南(2023年版). 胃肠病学. 2022(06): 341-375 . ![]() | |
18. | 汪涛,王学伟,蒋元烨,曹勤,季光. 162例药物性肝损伤患者分析. 肝脏. 2021(03): 243-246 . ![]() | |
19. | 王艳,王昱,王岚,田秋菊,杨瑞园,李柯鑫,刘立伟,王晓明,王宇,欧晓娟,贾继东,赵新颜. 中草药与西药致药物性肝损伤的临床特征及其预后的对比研究. 肝脏. 2021(04): 364-369 . ![]() | |
20. | 杨焕芝,李兴德,陈学平,张仲安,钱彦华,蒋潇,徐艳琼,宋沧桑. 93例药物性肝损伤患者临床特征分析. 中国药业. 2021(15): 122-125 . ![]() | |
21. | 何婷婷,王丽苹,任璐彤,崔延飞,柏兆方,郭玉明,宫嫚,王睿林. 中西药肝损伤临床及病理特征分析. 肝脏. 2021(09): 962-967 . ![]() | |
22. | 刘秀兰,李为,郭敏,杜金凤,刘东,李娟. 药物性肝损伤相关医疗损害案例分析. 药物流行病学杂志. 2021(11): 729-734 . ![]() | |
23. | 陈凯霞,赵广玉,印登阳,陈宏俊,黄继勋. 三种保肝药治疗化疗引起的DILI的药物经济学评价. 中国实用医药. 2021(35): 188-191 . ![]() | |
24. | 罗琼,朱哿瑞,顾宏图,刘坤,陈高峰,邢枫,陶艳艳,刘成海. 50例中草药与西药致药物性肝损伤患者的肝组织病理学特点比较. 临床肝胆病杂志. 2020(03): 596-601 . ![]() | |
25. | 蔡春梅. 妊娠早期肝功能异常60例的病因分析及干预. 中国城乡企业卫生. 2020(06): 100-102 . ![]() | |
26. | 周力. 中西药所致药物性肝损伤临床及病理特点研究. 临床医药文献电子杂志. 2020(39): 56 . ![]() | |
27. | 纪童童,陆海英,谭宁,于岩岩,徐小元. 急、慢性药物性肝损伤临床特征的对比分析. 临床肝胆病杂志. 2020(07): 1556-1561 . ![]() | |
28. | 何文昌,张克恭,赵凡惠,郭芮杉,高禄化,黄长形,王临旭. 290例药物性肝损伤患者临床特征分析. 实用肝脏病杂志. 2020(04): 540-543 . ![]() | |
29. | 李梁,陶应敏,张学敏,谢娟,陈园. 药物性肝损伤患者外周血清HIF1α和COX-2定量检测的临床价值. 肝脏. 2020(08): 848-852 . ![]() | |
30. | 李卉,张宏亮,杨天燕,黄振光,刘滔滔,韦芳. 我院125例抗结核药物不良反应报告分析. 中国医药导报. 2020(29): 151-154 . ![]() | |
31. | 姜正艳,郑亮. 异甘草酸镁治疗药物性肝损伤临床疗效观察. 世界最新医学信息文摘. 2019(07): 123 . ![]() | |
32. | 张惠娟,史祖宣,赵兰芳,高天慧,李健. 56例抗肿瘤药物致肝损伤临床特点分析. 临床肝胆病杂志. 2019(03): 574-578 . ![]() | |
33. | 梁栋,董晓锋,张燕,朱晓红,王建斌,刘妲妲,柳伟伟,方圆,王全楚. 98例药物性肝损伤的临床特点及诊疗体会. 肝脏. 2019(04): 389-391 . ![]() | |
34. | 黄波,张炜. 喜炎平注射液联合还原型谷胱甘肽治疗重症烧伤后急性肝功能损伤临床研究. 创伤与急危重病医学. 2019(03): 156-159 . ![]() | |
35. | 郭立杰,张海丛,叶立红,王超,杜婧,伍彦辉. 中西药所致药物性肝损伤临床及病理特点分析. 临床误诊误治. 2019(08): 28-33 . ![]() | |
36. | 吴宇宇,袁苏榆,孙四珍,王岁晶,丁洋. 药物性肝损伤诊断治疗进展概述. 药物流行病学杂志. 2018(08): 550-555 . ![]() | |
37. | 王建青,叶珺,郜玉峰,杨利琦. 106例药物性肝损伤患者用药回顾性调查. 中国医院药学杂志. 2018(18): 1967-1970 . ![]() |