PDF(Pubmed)
Abstract：
Autoimmune hepatitis (AIH) is a chronic inflammatory liver disease that can lead to cirrhosis and liver failure. AIH can present in all ages, races, and ethnicities, but it predominantly affects women. As a heterogeneous disease, AIH presents variably in different patients, making diagnosis and treatment a challenge. Currently, the standard treatment for AIH comprises immunosuppressants; however, their long-term use is associated with adverse effects. The pathogenesis of AIH is complex, involving T cells, macrophages, and plasma cells that invade the periportal parenchyma and lead to an inflammatory cascade that can result in liver damage. Due to the complexity of AIH pathogenesis, treatment targets several inflammatory pathways. However, unlike other autoimmune diseases in which targeted treatments have been approved, there has been little progress made in advancing the treatment paradigm for AIH. Major obstacles to progress include challenges in conducting clinical trials, particularly patient recruitment and ensuring a diverse range of backgrounds; poorly defined outcomes to assess treatment response and improved quality of life; and a lack of study designs that account for the stage of disease and variations in treatment. A focus on individualized and steroid-free treatment approaches is needed to improve AIH prognosis and minimize steroid-associated adverse effects.
摘要：
自身免疫性肝炎（AIH）是一种慢性炎症性肝病，可导致肝硬化和肝功能衰竭。AIH可以存在于所有年龄段，种族,和种族，但它主要影响女性。作为一种异质性疾病，AIH在不同的患者中表现不同，使诊断和治疗成为挑战。目前,AIH的标准治疗包括免疫抑制剂；然而，它们的长期使用与副作用有关。AIH的发病机制复杂,涉及T细胞，巨噬细胞，和浆细胞侵入门静脉周围实质并导致可导致肝损伤的炎症级联反应。由于AIH发病机制的复杂性,治疗靶向几种炎症途径。然而,与其他已批准靶向治疗的自身免疫性疾病不同，在推进AIH治疗模式方面进展甚微.取得进展的主要障碍包括进行临床试验的挑战,特别是患者招募和确保不同背景;评估治疗反应和生活质量的结局定义不明确;缺乏考虑疾病阶段和治疗差异的研究设计.需要关注个性化和无类固醇治疗方法，以改善AIH预后并最大程度地减少与类固醇相关的不良反应。
