Details

Autor(en) / Beteiligte

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

Titel

Etiopathogenesis of autoimmune hepatitis

Ist Teil von

• Journal of autoimmunity, 2018-12, Vol.95, p.133-143

Ort / Verlag

England: Elsevier Ltd

Erscheinungsjahr

2018

Quelle

Alma/SFX Local Collection

Beschreibungen/Notizen

• 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. •The etiology of AIH is unknown, but genetic and environmental factors are likely to play an important role.•GWAS studies demonstrated that AIH is mostly associated with HLA genes, although several non-HLA loci have been identified.•Several viruses have been linked to AIH, supporting the hypothesis of molecular mimicry between viruses and host components.•Microbiome serves as a reservoir of antigens that can initiate, maintain and/or perpetuate the autoimmune response in AIH.