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Pathogenesis and Clinical Practice in Gastroenterology pp 191–207Cite as

Immunology of primary biliary cirrhosis and primary sclerosing cholangitis

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Part of the book series: Falk Symposium ((FASS,volume 160))

Abstract

Primary biliary cirrhosis (PBC) is a chronic cholestatic liver disease of unknown aetiology characterized by high titre serum antimitochondrial autoantibodies (AMA), a striking female predominance, and an autoimmune-mediated destruction of the small and medium-size intrahepatic bile ducts1. PBC is a peculiar, yet representative, organ-specific autoimmune disease. The presence of serum AMA and autoreactive T and B cells, in conjunction with the co-occurrence of other autoimmune diseases, implies an autoimmune pathogenesis for PBC. A complex genetic background is suggested by the incidence of the disease among first-degree relatives2, the high concordance rate among twins3, and the proposed role for sex chromosome defects4. To summarize, PBC onset recognizes two necessary components: i.e. a permissive genetic background and an environmental trigger.

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Authors and Affiliations

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

    M. E. Gershwin

Authors
  1. A. Lleo
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  2. C. Selmi
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  3. P. Invernizzi
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  4. M. Podda
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  5. M. E. Gershwin
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Editors and Affiliations

  1. Department of Gastroenterology, Medical Centre Ljubljana, Slovenia

    I. Ferkolj

  2. University of Vienna, Vienna, Austria

    A. Gangl

  3. University of Mainz, Mainz, Germany

    P. R. Galle

  4. University Hospital Rebro, Zagreb, Croatia

    B. Vucelic

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© 2008 Springer and Falk Foundation e.V.

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Lleo, A., Selmi, C., Invernizzi, P., Podda, M., Gershwin, M.E. (2008). Immunology of primary biliary cirrhosis and primary sclerosing cholangitis. In: Ferkolj, I., Gangl, A., Galle, P.R., Vucelic, B. (eds) Pathogenesis and Clinical Practice in Gastroenterology. Falk Symposium, vol 160. Springer, Dordrecht. https://doi.org/10.1007/978-1-4020-8767-7_19

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  • DOI: https://doi.org/10.1007/978-1-4020-8767-7_19

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