The clinical phenotypes of autoimmune hepatitis

A comprehensive review

Qixia Wang, Fan Yang, Qi Miao, Edward L. Krawitt, M. Eric Gershwin, Xiong Ma

Research output: Contribution to journalArticle

46 Citations (Scopus)

Abstract

Autoimmune hepatitis (AIH) fulfills the generally accepted contemporary criteria of an autoimmune liver disease: the presence of autoantibodies and autoreactive T cells, a female gender bias, association with other autoimmune diseases, response to immunosuppressive therapy and strong associations with the major histocompatibility complex HLA loci. It occurs worldwide in both children and adults and is marked by both etiopathogenic and clinical heterogeneity, differing from the other putative autoimmune liver diseases, primary biliary cirrhosis (PBC) and primary sclerosing cholangitis (PSC), albeit occasionally presenting with overlapping features of PBC or PSC. Although diagnostic criteria have been established and validated, there are still major issues to be clarified due to its variability, such as autoantibody-negative AIH, drug-induced AIH, AIH sharing features with PBC or PSC, and post-transplant de novo AIH. In view of the diverse presentations and courses, including classical chronic onset, acute and acute severe onset, cirrhosis and decompensated cirrhosis, individualized management of patients is indicated. Each patient should receive a personalized analysis of the benefits and side effect risks of drugs. Herein we describe a comprehensive review of the clinical phenotypes of AIH underscoring its clinical heterogeneity.

Original languageEnglish (US)
Pages (from-to)98-107
Number of pages10
JournalJournal of Autoimmunity
Volume66
DOIs
StatePublished - Jan 1 2016

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Autoimmune Hepatitis
Phenotype
Sclerosing Cholangitis
Biliary Liver Cirrhosis
Autoimmune Diseases
Autoantibodies
Liver Diseases
Fibrosis
Chemical and Drug Induced Liver Injury
Sexism
Immunosuppressive Agents
Major Histocompatibility Complex
Drug-Related Side Effects and Adverse Reactions
Autoimmunity
T-Lymphocytes
Transplants

Keywords

  • Acute hepatitis
  • Autoantibodies
  • Autoimmune hepatitis
  • Chronic hepatitis
  • Drug-induced liver injury
  • Jaundice
  • Liver failure
  • Liver transplantation

ASJC Scopus subject areas

  • Immunology
  • Immunology and Allergy

Cite this

The clinical phenotypes of autoimmune hepatitis : A comprehensive review. / Wang, Qixia; Yang, Fan; Miao, Qi; Krawitt, Edward L.; Gershwin, M. Eric; Ma, Xiong.

In: Journal of Autoimmunity, Vol. 66, 01.01.2016, p. 98-107.

Research output: Contribution to journalArticle

Wang, Qixia ; Yang, Fan ; Miao, Qi ; Krawitt, Edward L. ; Gershwin, M. Eric ; Ma, Xiong. / The clinical phenotypes of autoimmune hepatitis : A comprehensive review. In: Journal of Autoimmunity. 2016 ; Vol. 66. pp. 98-107.
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