Cellular and Molecular Mechanisms of Autoimmune Hepatitis

G. J. Webb, G. M. Hirschfield, E. L. Krawitt, M. Eric Gershwin

Research output: Contribution to journalArticle

12 Citations (Scopus)

Abstract

Autoimmune hepatitis is an uncommon idiopathic syndrome of immune-mediated destruction of hepatocytes, typically associated with autoantibodies. The disease etiology is incompletely understood but includes a clear association with human leukocyte antigen (HLA) variants and other non-HLA gene variants, female sex, and the environment. Pathologically, there is a CD4 T cell-rich lymphocytic inflammatory infiltrate with variable hepatocyte necrosis and subsequent hepatic fibrosis. Attempts to understand pathogenesis are informed by several monogenetic syndromes that may include autoimmune liver injury, by several drug and environmental agents that have been identified as triggers in a minority of cases, by human studies that point toward a central role for CD4 effector and regulatory T cells, and by animal models of the disease. Nonspecific immunosuppression is the current standard therapy. Further understanding of the disease's cellular and molecular mechanisms may assist in the design of better-targeted therapies, aid the limitation of adverse effects from therapy, and inform individualized risk assessment and prognostication.

Original languageEnglish (US)
Pages (from-to)247-292
Number of pages46
JournalAnnual Review of Pathology: Mechanisms of Disease
Volume13
DOIs
StatePublished - Jan 24 2018

Fingerprint

Autoimmune Hepatitis
HLA Antigens
Hepatocytes
Animal Disease Models
Liver
Regulatory T-Lymphocytes
Autoantibodies
Immunosuppression
Fibrosis
Necrosis
Therapeutics
T-Lymphocytes
Wounds and Injuries
Pharmaceutical Preparations
Genes

Keywords

  • Autoantibodies
  • Autoimmune hepatitis
  • Autoimmunity
  • Cirrhosis
  • Hepatocyte
  • T cells

ASJC Scopus subject areas

  • Pathology and Forensic Medicine

Cite this

Cellular and Molecular Mechanisms of Autoimmune Hepatitis. / Webb, G. J.; Hirschfield, G. M.; Krawitt, E. L.; Gershwin, M. Eric.

In: Annual Review of Pathology: Mechanisms of Disease, Vol. 13, 24.01.2018, p. 247-292.

Research output: Contribution to journalArticle

Webb, G. J. ; Hirschfield, G. M. ; Krawitt, E. L. ; Gershwin, M. Eric. / Cellular and Molecular Mechanisms of Autoimmune Hepatitis. In: Annual Review of Pathology: Mechanisms of Disease. 2018 ; Vol. 13. pp. 247-292.
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