Heneghan Michael A, Lohse Ansgar Wilhelm
Institute of Liver Studies, King's College Hospital, London, United Kingdom.
I. Department of Medicine, University Medical Centre Hamburg-Eppendorf, Hamburg, Germany; European Reference Network on Hepatological Diseases (ERN RARE-LIVER), Germany.
J Hepatol. 2025 May;82(5):926-937. doi: 10.1016/j.jhep.2024.12.041. Epub 2025 Jan 27.
Autoimmune hepatitis (AIH) is an enigmatic, relatively rare disease with a variable spectrum of presentation whose pathogenesis, diagnosis and management remain a major challenge. We have performed a review of recent developments in basic science, epidemiology, clinical science, therapeutics, and regulatory science, evaluating the challenges associated with the application of translational research and clinical trial design to a condition that is a chameleon in nature, where outcomes range from relatively benign disease through cirrhosis and acute liver failure. This review is focused on developments from 2020 onwards so we can present a forward-looking view on the challenges and remaining questions that must be addressed to improve patient care and outcomes in AIH. We also outline areas of debate and offer insights into these areas.
自身免疫性肝炎（AIH）是一种神秘且相对罕见的疾病，其临床表现多样，发病机制、诊断和管理仍然是一项重大挑战。我们对基础科学、流行病学、临床科学、治疗学和监管科学的最新进展进行了综述，评估了将转化研究和临床试验设计应用于这种本质上像变色龙一样的疾病所面临的挑战，该疾病的结局从相对良性的疾病到肝硬化和急性肝衰竭不等。本综述聚焦于2020年以来的进展，以便我们能够对改善AIH患者护理和结局必须解决的挑战和遗留问题提出前瞻性观点。我们还概述了争议领域并对这些领域提供见解。
