Characterization of animal models for primary sclerosing cholangitis (PSC)

J Hepatol. 2014 Jun;60(6):1290-303. doi: 10.1016/j.jhep.2014.02.006. Epub 2014 Feb 19.


Primary sclerosing cholangitis (PSC) is a chronic cholangiopathy characterized by biliary fibrosis, development of cholestasis and end stage liver disease, high risk of malignancy, and frequent need for liver transplantation. The poor understanding of its pathogenesis is also reflected in the lack of effective medical treatment. Well-characterized animal models are utterly needed to develop novel pathogenetic concepts and study new treatment strategies. Currently there is no consensus on how to evaluate and characterize potential PSC models, which makes direct comparison of experimental results and effective exchange of study material between research groups difficult. The International Primary Sclerosing Cholangitis Study Group (IPSCSG) has therefore summarized these key issues in a position paper proposing standard requirements for the study of animal models of PSC.

Keywords: Animal model; Bile acids; Biliary fibrosis; Cholangiopathies; Cholestatic liver disease; Primary sclerosing cholangitis.

Publication types

  • Review

MeSH terms

  • Animals
  • Cholangitis, Sclerosing* / pathology
  • Cholangitis, Sclerosing* / physiopathology
  • Cholangitis, Sclerosing* / therapy
  • Disease Models, Animal*
  • Humans