自身免疫性肝炎(AIH)是一种以免疫介导的肝细胞破坏为特征的慢性肝病,导致炎症和纤维化。近年来,在理解发病机制方面取得了重大进展,流行病学,诊断,AIH的治疗。这项全面的叙述审查旨在提供这些进展的最新概述。回顾首先概述了AIH的历史背景,可以追溯到它在1940年代的最初认可,并强调了基于自身抗体谱的诊断标准和分类的演变。探索了AIH的流行病学,讨论其在不同地区的不同患病率以及遗传易感性的作用,病毒感染,和药物暴露作为危险因素。此外,本综述深入研究了AIH的发病机制,关注失调的免疫反应,T细胞的参与,和肠道微生物组的潜在贡献。临床表现,诊断标准,和肝活检作为诊断的关键工具也进行了讨论。关于管理,该综述对涉及糖皮质激素和硫唑嘌呤的标准一线治疗进行了深入分析,以及无反应病例的替代疗法。此外,研究了新兴的二线和三线治疗方案。总之,这篇叙述性综述强调了AIH的复杂性,并强调了早期诊断和个体化治疗方法对改善患者预后的重要性.需要进一步的研究和临床试验来优化AIH管理并确保受影响个体的长期预后。
Autoimmune hepatitis (AIH) is a chronic liver disease characterized by immune-mediated destruction of hepatocytes, leading to inflammation and fibrosis. In recent years, significant advances have been made in understanding the pathogenesis, epidemiology, diagnosis, and treatment of AIH. This comprehensive narrative
review aims to provide an up-to-date overview of these advances. The
review begins by outlining the historical background of AIH, dating back to its initial recognition in the 1940s, and highlights the evolution of diagnostic criteria and classification based on autoantibody profiles. The epidemiology of AIH is explored, discussing its varying prevalence across different regions and the role of genetic predisposition, viral infections, and drug exposure as risk factors. Furthermore, the
review delves into the pathogenesis of AIH, focusing on the dysregulated immune response, involvement of T cells, and potential contribution of the gut microbiome. Clinical presentation, diagnostic criteria, and liver biopsy as crucial tools for diagnosis are also discussed. Regarding management, the
review provides an in-depth analysis of the standard first-line treatments involving glucocorticoids and azathioprine, as well as alternative therapies for non-responsive cases. Additionally, emerging second and third-line treatment options are examined. In conclusion, this narrative
review highlights the complexity of AIH and underscores the importance of early diagnosis and individualized treatment approaches to improve patient outcomes. Further research and clinical trials are needed to optimize AIH management and ensure a better long-term prognosis for affected individuals.