Animal Models of Autoimmune Liver Diseases: a Comprehensive Review

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 o...

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Bibliographic Details
Published in:Clinical reviews in allergy & immunology 2020-04, Vol.58 (2), p.252-271
Main Authors: Liu, Shou-Pei, Bian, Zhen-Hua, Zhao, Zhi-Bin, Wang, Jinjun, Zhang, Weici, Leung, Patrick S.C., Li, Liang, Lian, Zhe-Xiong
Format: Article
Language:English
Subjects:
Allergology
Analysis
Animal models
B cells
Bile ducts
Cholangitis
Diagnosis
Health aspects
Hepatitis
Hepatocytes
Immunoglobulin G
Immunology
Infection
Internal Medicine
Liver diseases
Medicine
Medicine & Public Health
Microbiota
Prognosis
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Summary: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.
ISSN:1080-0549
1559-0267
DOI:10.1007/s12016-020-08778-6