Immunopathogenic Mechanisms of Autoimmune Hepatitis: How Much Do We Know from Animal Models?

Int J Mol Sci. 2016 Dec 1;17(12):2007. doi: 10.3390/ijms17122007.

Abstract

Autoimmune hepatitis (AIH) is characterized by a progressive destruction of the liver parenchyma and a chronic fibrosis. The current treatment of autoimmune hepatitis is still largely dependent on the administration of corticosteroids and cytostatic drugs. For a long time the development of novel therapeutic strategies has been hampered by a lack of understanding the basic immunopathogenic mechanisms of AIH and the absence of valid animal models. However, in the past decade, knowledge from clinical observations in AIH patients and the development of innovative animal models have led to a situation where critical factors driving the disease have been identified and alternative treatments are being evaluated. Here we will review the insight on the immunopathogenesis of AIH as gained from clinical observation and from animal models.

Keywords: AIH immunopathogenesis; T cells; autoantibodies; chemokines: CYP2D6 model; cytokines.

Publication types

  • Review

MeSH terms

  • Adrenal Cortex Hormones / therapeutic use
  • Animals
  • Autoantibodies / immunology
  • Cytochrome P-450 CYP2D6 / genetics
  • Cytochrome P-450 CYP2D6 / immunology
  • Disease Models, Animal
  • Fibrosis / drug therapy
  • Fibrosis / immunology*
  • Fibrosis / pathology
  • Hepatitis, Autoimmune / drug therapy*
  • Hepatitis, Autoimmune / genetics
  • Hepatitis, Autoimmune / immunology*
  • Hepatitis, Autoimmune / pathology
  • Humans
  • Liver / drug effects
  • Liver / pathology*
  • Mice
  • T-Lymphocytes / immunology

Substances

  • Adrenal Cortex Hormones
  • Autoantibodies
  • Cytochrome P-450 CYP2D6