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Primary Biliary Cirrhosis and Autoimmune Cholangitis

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Liver Immunology

Abstract

Primary biliary cirrhosis (PBC) is a chronic cholestatic liver disease of unknown etiology characterized by hightiter serum antimitochondrial autoantibodies (AMAs) and an autoimmune-mediated destruction of the small and mediumsized intrahepatic bile ducts. From a clinical standpoint, PBC is a peculiar, yet representative, autoimmune disease (Table ). It affects women more frequently than men, with a female-to-male ratio of 9 to 1, and the average age at diagnosis is within the fifth and sixth decades of life, with exceptional cases described in pediatric ages. Epidemiological data indicate a geographical pattern of PBC prevalence and incidence rates, which are higher in northern countries (England, Scandinavia, northern United States). The diagnosis of PBC is made when two of three criteria are fulfilled, i.e., presence of serum AMAs, increased enzymes indicating cholestasis (i.e., alkaline phosphatase) for longer than 6 mo, and a compatible or diagnostic liver histology. Clinical symptoms include fatigue, pruritus, and jaundice. The progression of PBC varies widely for unknown reasons, as represented by certain patients remaining asymptomatic and others reaching liver failure at young ages.

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Authors and Affiliations

  1. Division of Internal Medicine, San Paolo School of Medicine, University of Milan, Milano, Italy

    Carlo Selmi MD, PhD & Ana Lleo MD

  2. Division of Rheumatology, Allergy and Clinical Immunology, University of California at Davis School of Medicine, Davis, CA

    Carlo Selmi MD, PhD & M. Eric Gershwin MD, FACP

  3. Division of Internal Medicine, Department of Medicine, Surgery and Dentistry, San Paolo School of Medicine, University of Milan, Milan, Italy

    Pietro Invernizzi MD, PhD

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  1. Carlo Selmi MD, PhD
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  2. Ana Lleo MD
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  3. Pietro Invernizzi MD, PhD
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  4. M. Eric Gershwin MD, FACP
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Editors and Affiliations

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

    M. Eric Gershwin MD, FACP

  2. Departments of Medicine and Surgery Baylor Liver Health, Baylor College of Medicine, Houston, TX

    John M. Vierling MD, FACP

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

    Michael P. Manns MD

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© 2007 Humana Press Inc., Totowa, NJ

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Selmi, C., Lleo, A., Invernizzi, P., Eric Gershwin, M. (2007). Primary Biliary Cirrhosis and Autoimmune Cholangitis. In: Gershwin, M.E., Vierling, J.M., Manns, M.P. (eds) Liver Immunology. Humana Press. https://doi.org/10.1007/978-1-59745-518-3_19

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  • DOI: https://doi.org/10.1007/978-1-59745-518-3_19

  • Publisher Name: Humana Press

  • Print ISBN: 978-1-58829-818-8

  • Online ISBN: 978-1-59745-518-3

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