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Primary Biliary Cirrhosis pp 53–63Cite as

Animal models of primary biliary cirrhosis

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Abstract

PBC fulfills four of the five criteria for an autoimmune disease (Table 1), but the etiology and immunopathogenesis of PBC are not well defined. A better understanding of the mechanisms responsible for bile duct destruction and hepatic fibrosis during chronic non-suppurative destructive cholangitis may lead to more effective medical therapies for this disease. However, several factors hamper the capacity to address these questions in patients. The onset of PBC is obscured by an initial preclinical/asymptomatic stage that lasts up to 20 years. Consequently, advanced stage III (fibrosis) or stage IV (cirrhosis) liver pathology is present in 50% of subjects at the time of diagnosis. In addition, the functions of T cells freshly isolated from established lesions cannot be assessed, since percutaneous liver biopsy specimens do not yield adequate numbers of cells for in-vitro studies. Also, whether the T cells cloned from the livers of patients with established PBC are representative of the primary T cells that initiate destruction of intrahepatic bile ducts and hepatic fibrosis in vivo is unclear. Moreover, MHC-matched or autologous intrahepatic bile duct cells are not available to directly examine how T cells mediate destruction of intrahepatic bile duct cells. These obstacles have led to increasing interest in animal models of PBC.

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Authors
  1. C. D. Howell
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  2. J. Li
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  3. W. Chen
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Editor information

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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© 1998 Springer Science+Business Media Dordrecht

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Howell, C.D., Li, J., Chen, W. (1998). Animal models 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_7

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  • DOI: https://doi.org/10.1007/978-94-011-4884-9_7

  • Publisher Name: Springer, Dordrecht

  • Print ISBN: 978-94-010-6047-9

  • Online ISBN: 978-94-011-4884-9

  • eBook Packages: Springer Book Archive

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