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Primary Sclerosing Cholangitis (PSC): Current Concepts in Biology and Strategies for New Therapy

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Biliary Disease

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Abstract

Primary sclerosing cholangitis (PSC) is characterised morphologically by fibro-inflammatory stricture formation and typified phenotypically by strong clinical association with inflammatory bowel disease. Although rare, PSC harbours a significant and disproportionate clinical need, in which ~50% of patients develop advancing disease necessitating transplantation. These unacceptably poor outcomes highlight a critical therapeutic shortfall in hepatology, whereby no existing intervention has been shown to improve transplant-free survival. The clinical course in PSC can be unpredictable; and advances in clinical practice highlight the breadth of disease heterogeneity, that exists, whilst equally providing a more individualised assessment of patient risk. These developments are paralleled by notable pathological discovery, wherein recognition of enteric dysbiosis, essential lymphocyte recruitment pathways, and mucosal immunogenicity have driven a resurgence in clinical trial activity for appropriately stratified patient populations.

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Authors and Affiliations

  1. National Institute for Health Research (NIHR) Birmingham and Centre for Liver Research, Institute of Immunology and Immunotherapy, University of Birmingham, Birmingham, B15 2TT, UK

    Palak J. Trivedi

  2. Norwegian PSC Research Center, Department of Transplantation Medicine, Division of Cancer Medicine, Surgery and Transplantation, Oslo University Hospital Rikshospitalet, Oslo, Norway

    Tom H. Karlsen

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  1. Centre for Liver Research NIHR Biomedical Research Unit, Institute of Immunology and Immunotherapy University of Birmingham, Birmingham, United Kingdom

    Gideon Hirschfield

  2. Centre for Liver Research NIHR Biomedical Research Unit, Institute of Immunology and Immunotherapy University of Birmingham, Birmingham, United Kingdom

    David Adams

  3. Centre for Liver Research NIHR Biomedical Research Unit, Institute of Immunology and Immunotherapy University of Birmingham, Birmingham, United Kingdom

    Evaggelia Liaskou

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Trivedi, P.J., Karlsen, T.H. (2017). Primary Sclerosing Cholangitis (PSC): Current Concepts in Biology and Strategies for New Therapy. In: Hirschfield, G., Adams, D., Liaskou, E. (eds) Biliary Disease. Springer, Cham. https://doi.org/10.1007/978-3-319-50168-0_9

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