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The Diagnosis and Classification of Immune-Mediated Biliary Diseases

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Liver Immunology

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Abstract

Immune-mediated biliary diseases are frequent causes of morbidity and mortality in all age groups, and encountered by clinicians worldwide. Despite many advances in the understanding of the pathogenesis of these diseases, diagnosis largely rests on making a syndromic classification interpreting findings (clinical, laboratory, immunologic, histologic) carefully and in a context-specific manner. Many biliary diseases have an immune-mediated component to them (e.g., biliary atresia, drug-induced liver injury, liver allograft rejection) but the two largest categories of disease are primary biliary cirrhosis (PBC) and primary sclerosing cholangitis (PSC). Both are autoimmune biliary diseases with distinct and non-overlapping presentations. PBC is diagnosed in patients with cholestasis who nearly always have circulating anti-mitochondrial antibodies or specific antinuclear antibody reactivity. PSC is a hepatobiliary manifestation of inflammatory bowel disease, more frequent in men, and associated with cholangiographic or histologic evidence of large bile duct cholangiopathy, in the absence of definable secondary insults. This chapter aims to introduce the reader to relevant themes in the diagnosis and classification of immune-mediated biliary diseases.

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Acknowledgements

Prof Stefan Hübscher, Dr. Rachel Brown, and Dr. Abid Karim for provision of histologic and serologic images for the figures (University of Birmingham/Queen Elizabeth Hospital, Birmingham). Dr. Laurie Paraskevi, Radiology, University of Toronto, for cholangiograms.

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Authors and Affiliations

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

    Gideon M. Hirschfield M.B., B.Chir., Ph.D., F.R.C.P.

Authors
  1. Gideon M. Hirschfield M.B., B.Chir., Ph.D., F.R.C.P.
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Correspondence to Gideon M. Hirschfield M.B., B.Chir., Ph.D., F.R.C.P. .

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Editors and Affiliations

  1. Division of Rheumatology, Allergy and Clinical Immunology, University of California School of Medicine, Davis, California, USA

    M. Eric Gershwin

  2. Medicine and Surgery, Baylor College of Medicine, Houston, Texas, USA

    John M. Vierling

  3. Department of Gastroenterology, Hepatology and Endocrinology, Medical School of Hannover, Germany

    Michael P. Manns

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Hirschfield, G.M. (2014). The Diagnosis and Classification of Immune-Mediated Biliary Diseases. In: Gershwin, M., Vierling, J., Manns, M. (eds) Liver Immunology. Springer, Cham. https://doi.org/10.1007/978-3-319-02096-9_9

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  • DOI: https://doi.org/10.1007/978-3-319-02096-9_9

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  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-319-02095-2

  • Online ISBN: 978-3-319-02096-9

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