The clinical phenotypes of autoimmune hepatitis: A comprehensive review

J Autoimmun. 2016 Jan:66:98-107. doi: 10.1016/j.jaut.2015.10.006. Epub 2015 Nov 21.


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.

Keywords: Acute hepatitis; Autoantibodies; Autoimmune hepatitis; Chronic hepatitis; Drug-induced liver injury; Jaundice; Liver failure; Liver transplantation.

Publication types

  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Adult
  • Anti-Inflammatory Agents / adverse effects
  • Anti-Inflammatory Agents / therapeutic use
  • Autoantibodies / blood
  • Autoantibodies / immunology
  • Child
  • Female
  • Hepatitis, Autoimmune / diagnosis*
  • Hepatitis, Autoimmune / drug therapy
  • Hepatitis, Autoimmune / immunology*
  • Humans
  • Immunosuppressive Agents / adverse effects
  • Immunosuppressive Agents / therapeutic use
  • Liver / immunology
  • Liver / pathology
  • Liver Transplantation / adverse effects
  • Phenotype
  • Sex Factors


  • Anti-Inflammatory Agents
  • Autoantibodies
  • Immunosuppressive Agents