Division of Gastroenterology, Department of Medicine and Surgery, Center for Autoimmune Liver Diseases, University of Milano-Bicocca, Monza, Monza-Brianza, Italy -
European Reference Network on Hepatological Diseases (ERN RARE-LIVER), San Gerardo Hospital, Monza, Monza-Brianza, Italy -
Minerva Gastroenterol (Torino). 2023 Mar;69(1):61-83. doi: 10.23736/S2724-5895.20.02805-6. Epub 2020 Dec 3.
Autoimmune hepatitis (AIH) is a rare autoimmune disease of the liver with many open questions as regards its etiopathogenesis, natural history and clinical management. The classical picture of AIH is chronic hepatitis with fluctuating elevation of serum transaminases and Immunoglobulin G levels, the presence of circulating autoantibodies and typical histological features. However, atypical presentations do occur and are not well captured by current diagnostic scores, with important consequences in terms of missed diagnoses and delayed treatments. AIH is treated with corticosteroids and immunosuppressive drugs but up to 40% of patients do not achieve full biochemical response and are at risk of progressing to cirrhosis and liver failure. Moreover, standard therapies are associated by significant side-effects which may impair the quality of life of patients living with AIH. However, advances in the understanding of the underlying immunology of AIH is raising the prospect of novel therapies and optimization of existing therapeutic approaches to reduce side-effect burdens and potentially restore immunological tolerance. In this review we outlined the clinical characteristics, etiopathogenesis and management of AIH and current challenges in the diagnosis and management of AIH and provided evidence underlying the evolution of diagnostic and clinical management protocols.
自身免疫性肝炎（AIH）是一种罕见的肝脏自身免疫性疾病，其发病机制、自然史和临床管理仍存在许多未解决的问题。AIH 的典型表现为慢性肝炎，血清转氨酶和 IgG 水平波动升高，存在循环自身抗体和典型的组织学特征。然而，也会出现不典型表现，目前的诊断评分并不能很好地捕捉到这些表现，这在漏诊和延迟治疗方面带来了重要的后果。AIH 的治疗方法是使用皮质类固醇和免疫抑制剂，但多达 40%的患者无法实现完全的生化缓解，并且有发展为肝硬化和肝功能衰竭的风险。此外，标准治疗方法伴随着显著的副作用，可能会影响 AIH 患者的生活质量。然而，对 AIH 潜在免疫学的理解的进展，为新型治疗方法的提出和现有治疗方法的优化提供了前景，以减轻副作用负担，并有可能恢复免疫耐受。在这篇综述中，我们概述了 AIH 的临床特征、发病机制和管理，以及 AIH 的诊断和管理方面当前的挑战，并提供了支持诊断和临床管理方案演变的证据。
