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Molecular considerations of primary biliary cirrhosis

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Primary Biliary Cirrhosis

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Abstract

Primary biliary cirrhosis is a destructive autoimmune disease of the intrahepatic bile ducts characterized by inflammation of the portal triads, fibrosis and the presence of antimitochondrial antibodies1,2. The typical patient with PBC is 40–60 years of age, female, with a clinical presentation of lethargy, pruritus that is debilitating and persistent, or mild jaundice. PBC is virtually the only autoimmune disease never reported in a pediatric population, and is uncommon before age 30, after which the incidence gradually rises with age. Increasingly, however, patients are being detected in the presymptomatic stage as a result of multiphasic laboratory screening tests because of elevations in serum alkaline phosphatase. The pathological changes in PBC are best thought of as an autoimmune cholangiolitis and the common scheme of histological grading of severity recognizes four stages3.

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Authors
  1. M. E. Gershwin
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  2. C. T. Migliaccio
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  3. J. Van De Water
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  4. R. L. Coppel
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Editors and Affiliations

  1. Mayo Clinic, 200 First Street SW, Rochester, MN, 55905, USA

    Keith D. Lindor (Professor of Medicine) (Professor of Medicine)

  2. The Toronto Hospital, Western Division, University of Toronto, 399 Bathurst Street, Wing 4-828, Toronto, ONT, M5T 2S8, Canada

    E. Jenny Heathcote (Professor of Medicine) (Professor of Medicine)

  3. Service d’Hepatologie et Gastroenterologie, AP — Hôpital Saint-Antoine, 184, rue Du Faubourg, Saint-Antoine, 75571, Paris, France

    Raoul Poupon (Professor of Medicine) (Professor of Medicine)

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Gershwin, M.E., Migliaccio, C.T., Van De Water, J., Coppel, R.L. (1998). Molecular considerations of primary biliary cirrhosis. In: Lindor, K.D., Heathcote, E.J., Poupon, R. (eds) Primary Biliary Cirrhosis. Springer, Dordrecht. https://doi.org/10.1007/978-94-011-4884-9_6

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  • DOI: https://doi.org/10.1007/978-94-011-4884-9_6

  • Publisher Name: Springer, Dordrecht

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