Autoimmune hepatitis is an immune-mediated disorder characterised by hypergammaglobulinaemia, autoantibodies, and interface hepatitis. The mainstay of treatment is non-specific immunosuppression, consisting of steroids with or without azathioprine. Although most patients respond satisfactorily to steroid and thiopurine-based treatment regimens, up to 40% relapse and 10% undergo liver transplantation. The cause of autoimmune hepatitis is unknown, but evidence implicates both genetic and environmental factors in its pathogenesis. An imbalance between effector and regulatory mechanisms leads to the breakdown of immune tolerance and the consequent development of an autoimmune attack. Signalling pathways that have been implicated in the pathogenesis of autoimmune hepatitis involve the proinflammatory cytokines interferon-γ, IL-12, tumour necrosis factor-α, IL-6, and IL-23. Numerical and functional defects of regulatory T cells have a permissive role that enables autoimmune liver injury to occur and persist. New therapeutic strategies are needed, with the aim of obtaining long-lasting disease remission without inducing non-specific immunosuppression and a focus on inhibiting the intrahepatic proinflammatory milieu or expanding the pool of regulatory T cells, or both.

自身免疫性肝炎是一种以高丙种球蛋白血症，自身抗体和界面性肝炎为特征的免疫介导的疾病。治疗的主要手段是非特异性免疫抑制，包括有或没有硫唑嘌呤的类固醇。尽管大多数患者对基于类固醇和硫嘌呤的治疗方案均满意，但高达40％的复发和10％的患者接受了肝移植。自身免疫性肝炎的病因尚不清楚，但有证据表明遗传和环境因素都与自身的发病机制有关。效应子和调节机制之间的不平衡导致免疫耐受性的破坏和随之而来的自身免疫攻击的发展。与自身免疫性肝炎发病机制有关的信号通路涉及促炎细胞因子干扰素-γ，IL-12，肿瘤坏死因子-α，IL-6和IL-23。调节性T细胞的数字和功能缺陷具有允许作用，使自身免疫性肝损伤发生并持续存在。需要新的治疗策略，目的是在不引起非特异性免疫抑制的情况下获得持久的疾病缓解，并着重于抑制肝内促炎环境或扩大调节性T细胞的库，或两者兼而有之。