Department of Gastroenterology and Hepatology, Radboud University Medical Center, Nijmegen, The Netherlands.
Department of Medicine, Section of Digestive Diseases, Yale School of Medicine, New Haven, Connecticut, USA.
Liver Int. 2023 Jul;43(7):1375-1384. doi: 10.1111/liv.15573. Epub 2023 Apr 10.
Autoimmune hepatitis (AIH) is a rare autoimmune liver disease that is characterised by a chronic inflammatory immune reaction directed against hepatocytes. The disease results in a substantial reduction in quality of life and potentially leads to liver-related complications or death. The International Autoimmune Hepatitis Group (IAIHG) initiated a series of research workshops to uncover the scientific gaps and opportunities in AIH. This review summarises the results of the latest workshop in Maastricht in 2022 and reviews the current challenges in adult AIH, particularly in relation to four important aspects of AIH: diagnostics; new immunomodulatory therapies; clinical trial design; and unmet clinical needs. This review also summarises the progress made since the AIH workshop in 2017. Patients and patient representatives were actively involved in the parallel working groups alongside clinicians and researchers. Despite 40 years of experience with diagnosing and treating AIH, false diagnoses occur and treatment is still based on nonselective immunosuppression. In addition to the need for more specific diagnostic tests, prognostic markers and tailor-based treatments, a major unmet clinical need was identified in areas of care delivery and health-related quality of life.
自身免疫性肝炎(AIH)是一种罕见的自身免疫性肝病,其特征是针对肝细胞的慢性炎症免疫反应。该疾病导致生活质量大幅下降,并可能导致与肝脏相关的并发症或死亡。国际自身免疫性肝炎小组(IAIHG)发起了一系列研究研讨会,以揭示 AIH 中的科学差距和机会。本综述总结了 2022 年在马斯特里赫特举行的最新研讨会的结果,并回顾了成人 AIH 的当前挑战,特别是在 AIH 的四个重要方面:诊断;新型免疫调节疗法;临床试验设计;以及未满足的临床需求。本综述还总结了自 2017 年 AIH 研讨会以来取得的进展。患者及其代表与临床医生和研究人员一起积极参与了平行工作组。尽管在诊断和治疗 AIH 方面已有 40 年的经验,但仍会出现误诊,且治疗仍基于非选择性免疫抑制。除了需要更具体的诊断测试、预后标志物和基于个体化的治疗方法外,在护理提供和与健康相关的生活质量方面还确定了一个主要的未满足的临床需求。