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Terziroli Beretta-Piccoli B, Mieli-Vergani G, Vergani D. Autoimmmune hepatitis. Cell Mol Immunol. 2021;doi: 10.1038/s41423-021-00768-8.
Terziroli Beretta-Piccoli, B., Mieli-Vergani, G., & Vergani, D. (2021). Autoimmmune hepatitis. Cellular & molecular immunology, . https://doi.org/10.1038/s41423-021-00768-8
Terziroli Beretta-Piccoli, Benedetta, et al. "Autoimmmune hepatitis." Cellular & molecular immunology vol. (2021). doi: https://doi.org/10.1038/s41423-021-00768-8
Terziroli Beretta-Piccoli B, Mieli-Vergani G, Vergani D. Autoimmmune hepatitis. Cell Mol Immunol. 2021 Sep 27; doi: 10.1038/s41423-021-00768-8. Epub 2021 Sep 27. PMID: 34580437; PMCID: PMC8475398.
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Cell Mol Immunol. 2021 Sep 27; doi: 10.1038/s41423-021-00768-8. Epub 2021 Sep 27.

Autoimmmune hepatitis.

Cellular & molecular immunology

Benedetta Terziroli Beretta-Piccoli, Giorgina Mieli-Vergani, Diego Vergani

Affiliations

  1. Epatocentro Ticino & Facoltà di Scienze Biomediche, Università della Svizzera Italiana, Lugano, Switzerland. [email protected].
  2. Institute for Research in Biomedicine, Bellinzona, Switzerland. [email protected].
  3. King's College London Faculty of Life Sciences & Medicine at King's College Hospital, London, UK. [email protected].
  4. King's College London Faculty of Life Sciences & Medicine at King's College Hospital, London, UK.
  5. Paediatric Liver, GI and Nutrition Centre, MowatLabs, King's College Hospital, London, UK.
  6. Institute of Liver Studies, MowatLabs, King's College Hospital, London, UK.

PMID: 34580437 PMCID: PMC8475398 DOI: 10.1038/s41423-021-00768-8

Abstract

Autoimmune hepatitis (AIH) is a T-cell mediated, inflammatory liver disease affecting all ages and characterized by female preponderance, elevated serum transaminase and immunoglobulin G levels, positive circulating autoantibodies, and presence of interface hepatitis at liver histology. AIH type 1, affecting both adults and children, is defined by positive anti-nuclear and/or anti-smooth muscle antibodies, while type 2 AIH, affecting mostly children, is defined by positive anti-liver-kidney microsomal type 1 and/or anti-liver cytosol type 1 antibody. While the autoantigens of type 2 AIH are well defined, being the cytochrome P4502D6 (CYP2D6) and the formiminotransferase cyclodeaminase (FTCD), in type 1 AIH they remain to be identified. AIH-1 predisposition is conferred by possession of the MHC class II HLA DRB1*03 at all ages, while DRB1*04 predisposes to late onset disease; AIH-2 is associated with possession of DRB1*07 and DRB1*03. The majority of patients responds well to standard immunosuppressive treatment, based on steroid and azathioprine; second- and third-line drugs should be considered in case of intolerance or insufficient response. This review offers a comprehensive overview of pathophysiological and clinical aspects of AIH.

© 2021. The Author(s).

Keywords: Autoimmune Hepatitis; Genetic Predisposition; Immunopathophysiology; Treatment

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