Etiopathogenesis of autoimmune hepatitis

Annarosa Floreani, Paula Restrepo-Jiménez, Maria Francesca Secchi, Sara De Martin, Patrick S Leung, Edward Krawitt, Christopher Bowlus, M. Eric Gershwin, Juan Manuel Anaya

Research output: Contribution to journalReview article

23 Scopus citations

Abstract

Autoimmune hepatitis is a chronic inflammatory liver disease characterized by hypergammaglobulinemia, the presence of autoantibodies, and inflammation within the liver, including lymphocytic infiltrates and interface hepatitis. Autoimmune hepatitis shows a female predominance and can present at any age and in any ethnicity. The disease is thought to be a consequence of a break of immune tolerance leading to an autoimmune process that induces liver injury. The self-attack is triggered by T-helper cell-mediated liver autoantigen recognition and B-cell production of autoantibodies, and is sustained by impaired regulatory T cells number and function. Superimposed on a genetic predisposition, infections and environmental factors have been studied as triggering factors for the disease. Allelic variants in the HLA locus have been associated with susceptibility; associations with single nucleotide polymorphisms within non-HLA genes have also been assessed. Several factors have been described as triggers of autoimmune responses in predisposed individuals, including infections, alcohol, vitamin D deficiency, and an altered composition of the intestinal microbiome. Importantly, drugs and herbal agents may trigger classical autoimmune hepatitis, or may induce a liver disease with autoimmune features. Interactions between female hormones and genetic factors have been hypothesized to play a role in autoimmunity, although the exact role for these factors has not been fully established. Herein we present a review of the etiology of autoimmune hepatitis including de novo autoimmune hepatitis post-liver transplantation as well as animal models for its study.

Original languageEnglish (US)
Pages (from-to)133-143
Number of pages11
JournalJournal of Autoimmunity
Volume95
DOIs
StatePublished - Dec 1 2018

Keywords

  • Autoantibodies
  • Autoimmune hepatitis
  • Genome-wide association studies
  • Hormones
  • Microbiome
  • Primary biliary cholangitis
  • Primary sclerosing cholangitis

ASJC Scopus subject areas

  • Immunology and Allergy
  • Immunology

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    Floreani, A., Restrepo-Jiménez, P., Secchi, M. F., De Martin, S., Leung, P. S., Krawitt, E., Bowlus, C., Gershwin, M. E., & Anaya, J. M. (2018). Etiopathogenesis of autoimmune hepatitis. Journal of Autoimmunity, 95, 133-143. https://doi.org/10.1016/j.jaut.2018.10.020