Etiopathogenesis of autoimmune hepatitis.

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.

• Authors: Annarosa Floreani, Paula Restrepo-Jiménez, Maria Francesca Secchi, Sara De Martin, Patrick S C Leung, Edward Krawitt, Christopher L Bowlus, M Eric Gershwin, Juan-Manuel Anaya
• Journal: Journal of autoimmunity (J Autoimmun) Vol. 95 Pg. 133-143 (12 2018) ISSN: 1095-9157 [Electronic] England
• PMID: 30385083 (Publication Type: Journal Article, Review)
• Copyright: Copyright © 2018. Published by Elsevier Ltd.

Chemical References

• Autoantibodies
• Autoantigens
• HLA Antigens

Topics

• Alcoholism (complications, diagnosis, genetics, immunology)
• Animals
• Autoantibodies (biosynthesis)
• Autoantigens (genetics, immunology)
• B-Lymphocytes (immunology, pathology)
• Communicable Diseases (complications, diagnosis, genetics, immunology)
• Gene-Environment Interaction
• Genetic Predisposition to Disease
• HLA Antigens (genetics, immunology)
• Hepatitis, Autoimmune (diagnosis, etiology, genetics, immunology)
• Humans
• Liver (immunology, pathology)
• Liver Cirrhosis, Biliary (diagnosis, etiology, genetics, immunology)
• Liver Transplantation (adverse effects)
• Sex Factors
• T-Lymphocytes, Helper-Inducer (immunology, pathology)
• T-Lymphocytes, Regulatory (immunology, pathology)
• Vitamin D Deficiency (complications, diagnosis, genetics, immunology)