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Mechanisms of tissue injury in autoimmune liver diseases

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Abstract

Autoimmune diseases affecting the liver are mainly represented by autoimmune hepatitis (AIH), primary biliary cirrhosis (PBC) and primary sclerosing cholangitis (PSC). The characteristic morphologic patterns of injury are a chronic hepatitis pattern of damage in AIH, destruction of small intrahepatic bile ducts in PBC and periductal fibrosis and inflammation involving larger bile ducts in PSC. The factors responsible for initiation and perpetuation of the injury in all the three autoimmune liver diseases are not understood completely but are likely to be environmental triggers on the background of genetic variation in immune regulation. In this review, we summarise the current understanding of the mechanisms underlying the breakdown of self-tolerance in autoimmune liver diseases.

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Authors and Affiliations

  1. NIHR Biomedical Research Unit in Liver Disease and Centre for Liver Research, Institute of Biomedical Research, University of Birmingham, Birmingham, UK, B15 2TT

    Evaggelia Liaskou & Gideon M. Hirschfield

  2. Division of Rheumatology, Allergy and Clinical Immunology, University of California at Davis School of Medicine, 451 Health Sciences Drive, Suite 6510, Davis, CA, 95616, USA

    M. Eric Gershwin

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  1. Evaggelia Liaskou
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  2. Gideon M. Hirschfield
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  3. M. Eric Gershwin
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This article is a contribution to the special issue on Mechanisms of Tissue Injury in Autoimmune Diseases - Guest Editor: Dan Eilat

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Liaskou, E., Hirschfield, G.M. & Gershwin, M.E. Mechanisms of tissue injury in autoimmune liver diseases. Semin Immunopathol 36, 553–568 (2014). https://doi.org/10.1007/s00281-014-0439-3

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