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Autoimmune Hepatitis

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Liver Immunology

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Abstract

Autoimmune hepatitis (AIH) is a progressive inflammatory hepatopathy and an important cause of end-stage liver disease. Its aetiology remains unknown, though both genetic and environmental factors are involved in its development. The major mechanism of autoimmune liver damage involves immune reactions against host liver antigens. Numerical and functional defects of regulatory T cells play a permissive role, enabling autoimmune liver injury to occur and persist. The most typical features of AIH are female preponderance, hypergammaglobulinaemia, seropositivity for circulating autoantibodies and a picture of interface hepatitis on histology. Two types of AIH are distinguished according to serological profile: AIH type 1 patients are positive for anti-nuclear and/or anti-smooth muscle antibodies, whereas AIH type 2 patients are defined by the positivity for anti-liver kidney microsomal type 1 antibody and/or for anti-liver cytosol type 1 antibody. Clinical manifestations are variable, and its onset is often ill-defined, frequently mimicking acute hepatitis and its course may be fluctuating. AIH responds to immunosuppressive treatment in the majority of cases. Steroids with or without azathioprine should be instituted promptly upon diagnosis. Remission is achievable in 80% of patients. For the remaining 20% of patients, alternative immunosuppressive agents such as mycophenolate mofetil and calcineurin inhibitors are an option. A deeper understanding of the pathogenesis of AIH is likely to contribute to the development of novel treatments, such as the adoptive transfer of autologous expanded antigen-specific regulatory T cells.

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Authors and Affiliations

  1. Centro Hospitalar Sao Joao, Faculty of Medicine, Porto University, Porto, Portugal

    Rodrigo Liberal

  2. Paediatric Liver, GI and Nutrition Centre, Mowat Labs, Faculty of Life Sciences and Medicine at King’s College Hospital, London, UK

    Giorgina Mieli-Vergani

  3. Institute of Liver Studies, Mowat Labs, Faculty of Life Sciences and Medicine at King’s College Hospital, London, UK

    Diego Vergani

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  1. Rodrigo Liberal
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  2. Giorgina Mieli-Vergani
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  3. Diego Vergani
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Correspondence to Diego Vergani .

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Editors and Affiliations

  1. Division of Rheumatology, Allergy and Clinical Immunology, The University of California School of Medicine, Davis, CA, USA

    M. Eric Gershwin

  2. Departments of Medicine and Surgery, Baylor College of Medicine, Houston, TX, USA

    John M. Vierling

  3. Department of Medicine, Teikyo University School of Medicine, Itabashi, Tokyo, Japan

    Atsushi Tanaka

  4. President, Hannover Medical School, Hannover, Germany

    Michael P. Manns

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Liberal, R., Mieli-Vergani, G., Vergani, D. (2020). Autoimmune Hepatitis. In: Gershwin, M.E., M. Vierling, J., Tanaka, A., P. Manns, M. (eds) Liver Immunology . Springer, Cham. https://doi.org/10.1007/978-3-030-51709-0_26

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