自身免疫性肝病重叠综合征的诊断和治疗

姚尚娟; 李依琳; 陈明洁; 李强

doi:10.3969/j.issn.1001-5256.2023.08.028

自身免疫性肝病重叠综合征的诊断和治疗

DOI: 10.3969/j.issn.1001-5256.2023.08.028

山东大学附属公共卫生临床中心，济南 250012

基金项目:

山东省重点研发计划 (2021SFGC0504)

利益冲突声明：本文不存在任何利益冲突。

作者贡献声明：姚尚娟、李依琳、陈明洁负责收集数据，资料分析；姚尚娟负责撰写论文，修改文章；李强指导撰写文章，修改文章并最后定稿。

详细信息

通信作者:
李强，doctorliqiang@aliyun.com (ORCID: 0000-0002-8667-4676)

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- 文章访问数: 403
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- 被引次数: 0
出版历程
- 收稿日期: 2022-10-23
- 录用日期: 2022-11-25
- 出版日期: 2023-08-20

Advances in the diagnosis and treatment of autoimmune liver disease overlap syndrome

Shandong Public Health Clinical Center Affiliated to Shandong University, Jinan 250012, China

Research funding:

Key R&D plan of Shandong Province (2021SFGC0504)

More Information

Corresponding author: LI Qiang, doctorliqiang@aliyun.com (ORCID: 0000-0002-8667-4676)

摘要

摘要: 自身免疫性肝病重叠综合征是肝脏自身免疫性疾病的一种，肝硬化相关并发症发生率高、预后差。本文从流行病学、临床特点、诊断、治疗和预后等方面阐述了自身免疫性肝病重叠综合征的最新研究进展，重点总结了如何诊断并使用有效的方案治疗不同的重叠综合征，以期提高临床工作者对该类疾病的认识，及早的诊断和治疗该疾病。
- 自身免疫疾病 /
- 肝炎, 自身免疫性 /
- 诊断 /
- 治疗学
Abstract: Autoimmune liver disease overlap syndrome is one of the autoimmune diseases of the liver, and liver cirrhosis-related complications tend to have a high incidence rate and a poor prognosis. This article elaborates on the latest research advances in autoimmune liver disease overlap syndrome from the aspects of epidemiology, clinical characteristics, diagnosis, treatment, and prognosis and summarizes the diagnostic methods and effective treatment regimens for different types of overlap syndrome, so as to improve the understanding of such diseases among clinical workers and achieve timely diagnosis and treatment.
- Autoimune Diseases /
- Hepatitis, Autoimmune /
- Diagnosis /
- Therapeutics

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参考文献(46)

[1]	WANG G, TANAKA A, ZHAO H, et al. The Asian Pacific Association for the Study of the Liver clinical practice guidance: the diagnosis andmanagement of patients with autoimmune hepatitis[J]. Hepatol Int, 2021, 15(2): 223-257. DOI: 10.1007/s12072-021-10170-1.
[2]	LINDOR KD, BOWLUS CL, BOYER J, et al. Primary biliary cholangitis: 2018 practice guidance from the american association for the study of liver diseases[J]. Hepatology, 2019, 69(1): 394-419. DOI: 10.1002/hep.30145.
[3]	RABIEE A, SILVEIRA MG. Primary sclerosing cholangitis[J]. Transl Gastroenterol Hepatol, 2021, 6: 29. DOI: 10.21037/tgh-20-266.
[4]	LIU Y, HAN K, LIU C, et al. Clinical characteristics and prognosis of concomitant primary biliary cholangitis and autoimmune diseases: a retrospective study[J]. Can J Gastroenterol Hepatol, 2021, 2021: 5557814. DOI: 10.1155/2021/5557814.
[5]	KATSUMI T, UENO Y. Epidemiology and surveillance of autoimmune hepatitis in Asia[J]. Liver Int, 2022, 42(9): 2015-2022. DOI: 10.1111/liv.15155.
[6]	Chinese Societyof Hepatology, Chinese Medical Association. Guidelines on the diagnosis and management of autoimmune hepatitis (2021)[J]. J Clin Hepatol, 2022, 38(1): 42-49. DOI: 10.3760/cma.j.cn112138-20211112-00796. 中华医学会肝病学会. 自身免疫性肝炎诊断和治疗指南(2021)[J]. 临床肝胆病杂志, 2022, 38(1): 42-49. DOI: 10.3760/cma.j.cn112138-20211112-00796.
[7]	TRIVEDI PJ, HIRSCHFIELD GM. Recent advances in clinical practice: epidemiology of autoimmune liver diseases[J]. Gut, 2021, 70(10): 1989-2003. DOI: 10.1136/gutjnl-2020-322362.
[8]	Chinese Societyof Hepatology, Chinese Medical Association. Guidelines on the diagnosis and management of primary biliary cholangitis hepatitis(2021)[J]. J Clin Hepatol, 2022, 38(1): 35-41. DOI: 10.3760/cma.j.cn112138-20211112-00794. 中华医学会肝病学分会, 原发性胆汁性胆管炎的诊断和治疗指南(2021)[J]. 临床肝胆病杂志, 2022, 38(1): 35-41. DOI: 10.3760/cma.j.cn112138-20211112-00794.
[9]	LLEO A, JEPSEN P, MORENGHI E, et al. Evolving trends in female to male incidence and male mortality of primary biliary cholangitis[J]. Sci Rep, 2016, 6: 25906. DOI: 10.1038/srep25906.
[10]	GRIFFITHS L, DYSON JK, JONES DE. The new epidemiology of primary biliary cirrhosis[J]. Semin Liver Dis, 2014, 34(3): 318-328. DOI: 10.1055/s-0034-1383730.
[11]	YOU H, MA X, EFE C, et al. APASL clinical practice guidance: the diagnosis and management of patients with primary biliary cholangitis[J]. Hepatol Int, 2022, 16(1): 1-23. DOI: 10.1007/s12072-021-10276-6.
[12]	RAZAFINDRAZOTO CI, RAKOTOZAFINDRABE ALR, RANDRIAMIFIDY NH, et al. Primary biliary cholangitis-autoimmune hepatitis overlap syndrome: Case report and literature review[J]. Clin Case Rep, 2021, 9(3): 1647-1650. DOI: 10.1002/ccr3.3861.
[13]	ZHANG W, DE D, MOHAMMED KA, et al. New scoring classification for primary biliary cholangitis-autoimmune hepatitis overlap syndrome[J]. Hepatol Commun, 2018, 2(3): 245-253. DOI: 10.1002/hep4.1148.
[14]	PUROHIT T, CAPPELL MS. Primary biliary cirrhosis: Pathophysiology, clinical presentation and therapy[J]. World J Hepatol, 2015, 7(7): 926-941. DOI: 10.4254/wjh.v7.i7.926.
[15]	European Association for the Study of the Liver. EASL clinical practice guidelines: The diagnosis and management of patients with primary biliary cholangitis[J]. J Hepatol, 2017, 67(1): 145-172. DOI: 10.1016/j.jhep.2017.03.022.
[16]	WANG Q, SELMI C, ZHOU X, et al. Epigenetic considerations and the clinical reevaluation of the overlap syndrome between primary biliary cirrhosis and autoimmune hepatitis[J]. J Autoimmun, 2013, 41: 140-145. DOI: 10.1016/j.jaut.2012.10.004.
[17]	HENNES EM, ZENIYA M, CZAJA AJ, et al. Simplified criteria for the diagnosis of autoimmune hepatitis[J]. Hepatology, 2008, 48(1): 169-176. DOI: 10.1002/hep.22322.
[18]	BOBERG KM, CHAPMAN RW, HIRSCHFIELD GM, et al. Overlap syndromes: the International Autoimmune Hepatitis Group (IAIHG) position statement on a controversial issue[J]. J Hepatol, 2011, 54(2): 374-385. DOI: 10.1016/j.jhep.2010.09.002.
[19]	MACK CL, ADAMS D, ASSIS DN, et al. Diagnosis and management of autoimmune hepatitis in adults and children: 2019 practice guidance and guidelines from the American Association for the Study of Liver Diseases[J]. Hepatology, 2020, 72(2): 671-722. DOI: 10.1002/hep.31065.
[20]	WANG QX, YAN L, MA X. Autoimmune hepatitis in the Asia-Pacific area[J]. J Clin Transl Hepatol, 2018, 6(1): 48-56. DOI: 10.14218/JCTH.2017.00032.
[21]	CZAJA AJ. Diagnosis and management of the overlap syndromes of autoimmune hepatitis[J]. Can J Gastroenterol, 2013, 27(7): 417-423. DOI: 10.1155/2013/198070.
[22]	DURAZZO M, PREMOLI A, PASCHETTA E, et al. Overlap syndromes of autoimmune hepatitis: an open question[J]. Dig Dis Sci, 2013, 58(2): 344-348. DOI: 10.1007/s10620-012-2378-y.
[23]	JIANG Y, XU BH, RODGERS B, et al. Characteristics and inpatient outcomes of primary biliary cholangitis and autoimmune hepatitis overlap syndrome[J]. J Clin Transl Hepatol, 2021, 9(3): 392-398. DOI: 10.14218/JCTH.2021.00008.
[24]	ZHANG H, YANG J, ZHU R, et al. Combination therapy of ursodeoxycholic acid and budesonide for PBC-AIH overlap syndrome: a meta-analysis[J]. Drug Des Devel Ther, 2015, 9: 567-574. DOI: 10.2147/DDDT.S74515.
[25]	CHAZOUILLÈRES O, WENDUM D, SERFATY L, et al. Long term outcome and response to therapy of primary biliary cirrhosis-autoimmune hepatitis overlap syndrome[J]. J Hepatol, 2006, 44(2): 400-406. DOI: 10.1016/j.jhep.2005.10.017.
[26]	YOKOKAWA J, SAITO H, KANNO Y, et al. Overlap of primary biliary cirrhosis and autoimmune hepatitis: Characteristics, therapy, and long term outcomes[J]. J Gastroenterol Hepatol, 2010, 25(2): 376-382. DOI: 10.1111/j.1440-1746.2009.06018.x.
[27]	FAN X, ZHU Y, MEN R, et al. Efficacy and safety of immunosuppressive therapy for pbc-aih overlap syndrome accompanied by decompensated cirrhosis: a real-world study[J]. Can J Gastroenterol Hepatol, 2018, 2018: 1965492. DOI: 10.1155/2018/1965492.
[28]	D'AMATO D, de VINCENTIS A, MALINVERNO F, et al. Real-world experience with obeticholic acid in patients with primary biliary cholangitis[J]. JHEP Rep, 2021, 3(2): 100248. DOI: 10.1016/j.jhepr.2021.100248.
[29]	CASTRO LIMO JD, ROMERO-GUTIÉRREZ M, RUIZ MARTíN J. Effective treatment of autoimmune hepatitis-primary biliary cholangitis overlap syndrome with obeticholic acid[J]. Rev Esp Enferm Dig, 2020, 112(9): 737. DOI: 10.17235/reed.2020.6883/2020.
[30]	DHIMAN P, MALHOTRA S. Overlap syndromes: an emerging diagnostic and therapeutic challenge[J]. Saudi J Gastroenterol, 2014, 20(6): 342-349. DOI: 10.4103/1319-3767.145316.
[31]	YOSHIOKA Y, TANIAI M, HASHIMOTO E, et al. Clinical profile of primary biliary cirrhosis with features of autoimmune hepatitis: Importance of corticosteroid therapy[J]. Hepatol Res, 2014, 44(9): 947-955. DOI: 10.1111/hepr.12210.
[32]	ISAYAMA H, TAZUMA S, KOKUDO N, et al. Clinical guidelines for primary sclerosing cholangitis 2017[J]. J Gastroenterol, 2018, 53(9): 1006-1034. DOI: 10.1007/s00535-018-1484-9.
[33]	Chinese Societyof Hepatology, Chinese Medical Association. Guidelines on the diagnosis and management of primary sclerosing cholangitis hepatitis(2021)[J]. J Clin Hepatol, 2022, 38(1): 50-61. DOI: 10.3760/cma.j.cn112138-20211109-00786. 中华医学会肝病学分会, 原发性硬化性胆管炎诊断及治疗指南(2021)[J]. 临床肝胆病杂志, 2022, 38(1): 50-61. DOI: 10.3760/cma.j.cn112138-20211109-00786.
[34]	CHAPMAN R, FEVERY J, KALLOO A, et al. Diagnosis and management of primary sclerosing cholangitis[J]. Hepatology, 2010, 51(2): 660-678. DOI: 10.1002/hep.23294.
[35]	MAGO S, WU GY. Primary sclerosing cholangitis and primary biliary cirrhosis overlap syndrome: a review[J]. J Clin Transl Hepatol, 2020, 8(3): 336-346. DOI: 10.14218/JCTH.2020.00036.
[36]	SAICH R, CHAPMAN R. Primary sclerosing cholangitis, autoimmune hepatitis and overlap syndromes in inflammatory bowel disease[J]. World J Gastroenterol, 2008, 14(3): 331-337. DOI: 10.3748/wjg.14.331.
[37]	SARCOGNATO S, SACCHI D, GRILLO F, et al. Autoimmune biliary diseases: primary biliary cholangitis and primary sclerosing cholangitis[J]. Pathologica, 2021, 113(3): 170-184. DOI: 10.32074/1591-951X-245.
[38]	Chinese Society of Hepatology, Chinese Society of Gastroenterology & Chinese Society of Infectious Diseases. Chinese consensus on the diagnosis and management of autoimmune hepatitis (2015)[J]. J Clin Hepatol, 2016, 32(1): 9-22. DOI: 10.3969/j.issn.1001-5256.2016.01.002. 中华医学会肝病学分会, 中华医学会消化病学会, 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. DOI: 10.3969/j.issn.1001-5256.2016.01.002.
[39]	FREEDMAN BL, DANFORD CJ, PATWARDHAN V, BONDER A. Treatment of overlap syndromes in autoimmune liver disease: a systematic review and meta-analysis[J]. J Clin Med, 2020, 9(5): 1449. DOI: 10.3390/jcm9051449.
[40]	European Association for the Study of the Liver. EASL clinical practice guidelines: management of cholestatic liver diseases[J]. J Hepatol, 2009, 51(2): 237-267. DOI: 10.1016/j.jhep.2009.04.009.
[41]	FLOREANI A, RIZZOTTO ER, FERRARA F, et al. Clinical course and outcome of autoimmune hepatitis/primary sclerosing cholangitis overlap syndrome[J]. Am J Gastroenterol, 2005, 100(7): 1516-1522. DOI: 10.1111/j.1572-0241.2005.41841.x.
[42]	AL-CHALABI T, PORTMANN BC, BERNAL W, et al. Autoimmune hepatitis overlap syndromes: an evaluation of treatment response, long-term outcome and survival[J]. Aliment Pharmacol Ther, 2008, 28(2): 209-220. DOI: 10.1111/j.1365-2036.2008.03722.x.
[43]	BOONSTRA K, BEUERS U, PONSIOEN CY. Epidemiology of primary sclerosing cholangitis and primary biliary cirrhosis: a systematic review[J]. J Hepatol, 2012, 56(5): 1181-1188. DOI: 10.1016/j.jhep.2011.10.025.
[44]	JEEVAGAN A. Overlap of primary biliary cirrhosis and primary sclerosing cholangitis - a rare coincidence or a new syndrome[J]. Int J Gen Med, 2010, 3: 143-146. DOI: 10.2147/ijgm.s11201.
[45]	SUNDARAM S, S K, MAZUMDAR S, et al. Overlap syndrome between primary biliary cholangitis and primary sclerosing cholangitis[J]. ACG Case Rep J, 2018, 5: e54. DOI: 10.14309/crj.2018.54.
[46]	OLIVEIRA EM, OLIVEIRA PM, BECKER V, et al. Overlapping of primary biliary cirrhosis and small duct primary sclerosing cholangitis: first case report[J]. J Clin Med Res, 2012, 4(6): 429-433. DOI: 10.4021/jocmr1060w.