The immunogenetics of autoimmune cholestasis

Palak Trivedi, Gideon Hirschfield

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13 Citations (Scopus)
157 Downloads (Pure)


The immune-mediated hepatobiliary diseases, primary biliary cirrhosis and primary sclerosing cholangitis are relatively rare, albeit and account for a significant amount of liver transplant activity and liver-related mortality globally. Precise disease mechanisms are yet to be described although a contributory role of genetic predisposition is firmly established. In addition to links with the major histocompatibility complex, a number of associations outside this region harbor additional loci which underscore the fundamental role of breaks in immune tolerance and mucosal immunogenicity in the pathogenesis of autoimmune biliary disease. We provide an overview of these key discoveries before discussing putative avenues of therapeutic exploitation based on existing findings.
Original languageEnglish
Pages (from-to)15–31
JournalClinics in Liver Disease
Issue number1
Early online date20 Nov 2015
Publication statusPublished - Feb 2016


  • Autoimmunity
  • Autoimmune liver disease
  • Mucosal immunity
  • Primary sclerosing cholangitis
  • Primary biliary cirrhosis


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