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Liberal R, Vergani D, Mieli-Vergani G, et al. Update on Autoimmune Hepatitis. J Clin Transl Hepatol. 2015;3(1):42-52doi: 10.14218/JCTH.2014.00032.
Liberal, R., Vergani, D., Mieli-Vergani, G., & Downer, J. T. (2015). Update on Autoimmune Hepatitis. Journal of clinical and translational hepatology, 3(1), 42-52. https://doi.org/10.14218/JCTH.2014.00032
Liberal, Rodrigo, et al. "Update on Autoimmune Hepatitis." Journal of clinical and translational hepatology vol. 3,1 (2015): 42-52. doi: https://doi.org/10.14218/JCTH.2014.00032
Liberal R, Vergani D, Mieli-Vergani G, Downer JT. Update on Autoimmune Hepatitis. J Clin Transl Hepatol. 2015 Mar;3(1):42-52. doi: 10.14218/JCTH.2014.00032. Epub 2015 Mar 15. PMID: 26357634; PMCID: PMC4542083.
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J Clin Transl Hepatol. 2015 Mar;3(1):42-52. doi: 10.14218/JCTH.2014.00032. Epub 2015 Mar 15.

Update on Autoimmune Hepatitis.

Journal of clinical and translational hepatology

Rodrigo Liberal, Diego Vergani, Giorgina Mieli-Vergani, Downer

Affiliations

  1. Paediatric Liver, GI & Nutrition Centre and Institute of Liver Studies, King's College London School of Medicine at King's College Hospital, London, UK.

PMID: 26357634 PMCID: PMC4542083 DOI: 10.14218/JCTH.2014.00032

Abstract

Autoimmune hepatitis (AIH), a liver disorder affecting both children and adults, is characterized by inflammatory liver histology, elevated transaminase levels, circulating nonorganspecific autoantibodies, and increased levels of immunoglobulin G, in the absence of a known etiology. Two types of AIH are recognized according to seropositivity: smooth muscle antibody and/or antinuclear antibody define AIH type 1 and antibodies to liver-kidney microsome type 1 and/or liver cytosol type 1 define AIH type 2. AIH type 1 affects both adults and children, while AIH type 2 is mainly a paediatric disease, though it does occasionally affects young adults. AIH should be considered during the diagnostic workup of any patient with increased liver enzyme levels. AIH is exquisitely responsive to immunosuppressive treatment with prednisolone with or without azathioprine, with symptom free long-term survival for the majority of patients. For those who do not respond to standard treatment, or who are difficult-to-treat, mycophenolate mofetil and, in the absence of a response, calcineurin inhibitors should be tried in addition to steroids. The pathogenesis of AIH is not fully understood, although there is mounting evidence that genetic susceptibility, molecular mimicry and impaired immunoregulatory networks contribute to the initiation and perpetuation of the autoimmune attack. Liver damage is thought to be mediated primarily by CD4 T-cells, although recent studies support the involvement of diverse populations, including Th17 cells. 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 antigenspecific regulatory T-cells, which ultimately aim at restoring tolerance to liver-derived antigens.

Keywords: Autoantibodies; Autoimmune hepatitis; Immunogenetics; Immunosuppresion; Regulatory T cells

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