自身免疫性肝炎是什么?第五届国际自身免疫性肝炎组研究研讨会综述

IF 9.5 1区 医学 Q1 GASTROENTEROLOGY & HEPATOLOGY
Bastian Engel , David N. Assis , Mamatha Bhat , Jan Clusmann , Joost PH. Drenth , Alessio Gerussi , María-Carlota Londoño , Ye Htun Oo , Ida Schregel , Marcial Sebode , Richard Taubert , the International Autoimmune Hepatitis Group (IAIHG) collaborators, the European Reference Network for Rare Liver Diseases (ERN RARE-LIVER)
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引用次数: 0

摘要

自身免疫性肝炎(AIH)是一种罕见的慢性肝病,在许多国家发病率都在上升。对肝脏的慢性自身免疫反应可引起肝脏和肝外症状,如果治疗不当,生活质量下降和无肝移植存活率降低。虽然皮质类固醇和硫嘌呤的标准治疗可改善AIH患者的预期寿命,但缓解率和耐受性通常被高估,与风湿病或炎症性肠病相比,替代一线和补偿性治疗的发展令人失望地缓慢。其他差距包括缺乏AIH的疾病特异性诊断标记物。同样,药物诱导的自身免疫样肝炎的新实体强调需要重新评估以前的诊断标准。在过去十年中,国际aihg组织(IAIHG)发起了一系列研究研讨会,以促进发现研究差距,并随后通过刺激专家中心之间的合作来加快科学进步的步伐。本综述报告了2024年6月在德国汉诺威举行的第五届研究研讨会的成果,并总结了自2022年第四届研讨会以来取得的进展。来自欧洲参考网络(ERN)罕见肝青年小组的患者代表参加了研讨会。今年第五届研讨会的具体目标是:(1)进一步改善诊断。(2)启动肝外免疫介导疾病药物的临床试验,包括知识转移,包括B细胞消耗CAR - T细胞。(3)利用多组学方法提高对疾病发病机制的理解。(4)肿瘤学或移植医学中基于机器学习的方法的应用,以提高AIH的诊断和预后预测。
本文章由计算机程序翻译,如有差异,请以英文原文为准。

Quo vadis autoimmune hepatitis? - Summary of the 5th international autoimmune hepatitis group research workshop 2024

Quo vadis autoimmune hepatitis? - Summary of the 5th international autoimmune hepatitis group research workshop 2024
Autoimmune hepatitis (AIH) is a rare chronic liver disease with an increasing incidence in many countries. Chronic autoimmune responses against the liver can cause hepatic and extrahepatic symptoms, decreased quality of life and reduced liver transplant-free survival if inadequately treated. Although standard treatment with corticosteroids and thiopurines improves the life expectancy of patients with AIH, remission rates and tolerability are generally overestimated and the development of alternative first-line and salvage therapies has been disappointingly slow compared to in rheumatological diseases or inflammatory bowel disease. Other gaps include the lack of disease-specific diagnostic markers for AIH. Similarly, the new entity of drug-induced autoimmune-like hepatitis underscores the need to re-evaluate previous diagnostic criteria. The International AIH Group (IAIHG) has initiated a series of research workshops over the last decade to promote the identification of research gaps and subsequently improve the pace of scientific progress by stimulating collaboration between expert centres. This review reports on the results of the 5th Research Workshop, held in Hannover, Germany in June 2024, and summarises the progress made since the 4th Workshop in 2022. Patient representatives from the European Reference Network (ERN) Rare Liver Youth Panel participated in the workshop. The specific objectives of this year's 5th Workshop were: (1) To further improve diagnostics. (2) Initiate clinical trials including knowledge transfer on drugs from extrahepatic immune-mediated diseases, including B cell-depleting CAR T cells. (3) Utilisation of multi-omics approaches to improve the understanding of disease pathogenesis. (4) Application of machine learning-based approaches established in oncology or transplantation medicine to improve diagnosis and outcome prediction in AIH.
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来源期刊
JHEP Reports
JHEP Reports GASTROENTEROLOGY & HEPATOLOGY-
CiteScore
12.40
自引率
2.40%
发文量
161
审稿时长
36 days
期刊介绍: JHEP Reports is an open access journal that is affiliated with the European Association for the Study of the Liver (EASL). It serves as a companion journal to the highly respected Journal of Hepatology. The primary objective of JHEP Reports is to publish original papers and reviews that contribute to the advancement of knowledge in the field of liver diseases. The journal covers a wide range of topics, including basic, translational, and clinical research. It also focuses on global issues in hepatology, with particular emphasis on areas such as clinical trials, novel diagnostics, precision medicine and therapeutics, cancer research, cellular and molecular studies, artificial intelligence, microbiome research, epidemiology, and cutting-edge technologies. In summary, JHEP Reports is dedicated to promoting scientific discoveries and innovations in liver diseases through the publication of high-quality research papers and reviews covering various aspects of hepatology.
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