Autoimmune liver diseases (AILDs) are potentially life-threatening chronic liver diseases which include autoimmune hepatitis, primary biliary cholangitis, primary sclerosing cholangitis, and recently characterized IgG4-related sclerosing cholangitis. They are caused by immune attack on hepatocytes or bile ducts, with different mechanisms and clinical manifestations. The etiologies of AILDs include a susceptible genetic background, environment insults, infections, and changes of commensal microbiota, but remain complicated. Understanding of the underlying mechanisms of AILDs is mandatory for early diagnosis and intervention, which is of great importance for better prognosis. Thus, animal models are developed to mimic the pathogenesis, find biomarkers for early diagnosis, and for therapeutic attempts of AILDs. However, no animal models can fully recapitulate features of certain AILD, especially the late stages of diseases. Certain limitations include different living condition, cell composition, and time frame of disease development and resolution. Moreover, there is no IgG4 in rodents which exists in human. Nevertheless, the understanding and therapy of AILDs have been greatly advanced by the development and mechanistic investigation of animal models. This review will provide a comprehensive overview of traditional and new animal models that recapitulate different features and etiologies of distinct AILDs.

中文翻译：

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自身免疫性肝病动物模型：综合综述。

自身免疫性肝病（AILD）是潜在威胁生命的慢性肝脏疾病，包括自身免疫性肝炎，原发性胆管性胆管炎，原发性硬化性胆管炎，以及最近表征的IgG4相关性硬化性胆管炎。它们是由对肝细胞或胆管的免疫攻击引起的，具有不同的机制和临床表现。AILD的病因包括易感的遗传背景，环境侮辱，感染和共生菌群的变化，但仍然很复杂。了解AILD的潜在机制对于早期诊断和干预是必不可少的，这对于改善预后至关重要。因此，开发了动物模型来模拟发病机理，找到用于早期诊断和用于AILD的治疗尝试的生物标记。然而，没有动物模型能够完全概括某些AILD的特征，尤其是疾病的晚期。某些局限性包括不同的生活条件，细胞组成以及疾病发展和解决的时间框架。而且，在人类中不存在啮齿动物中的IgG4。然而，通过动物模型的发展和机理研究，极大地促进了对AILD的理解和治疗。这篇综述将提供传统动物模型和新动物模型的全面概述，概述了不同AILD的不同特征和病因。在人类中，啮齿动物中没有IgG4。然而，通过动物模型的发展和机理研究，极大地促进了对AILD的理解和治疗。这篇综述将提供传统动物模型和新动物模型的全面概述，概述了不同AILD的不同特征和病因。在人类中，啮齿动物中没有IgG4。然而，通过动物模型的发展和机理研究，极大地促进了对AILD的理解和治疗。这篇综述将提供传统动物模型和新动物模型的全面概述，概述了不同AILD的不同特征和病因。