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Pathogenesis of Primary Biliary Cirrhosis

Lookup NU author(s): Professor David Jones

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Abstract

Autoimmune phenomena have been recognized in primary biliary cirrhosis (PBC) for more than 50 years and the specificity of the characteristic responses directed at highly conserved mitochondrial antigens determined in detail over the past 20. Effecter autoreactive immune responses are characterized and potential mechanisms of breakdown of tolerance to self proposed. Elements of the clinical pattern of PBC, including the recurrence of the disease across HLA boundaries after liver transplantation, remain difficult to reconcile with a simple autoimmune model. Alternative (but not necessary mutually exclusive) pathogenetic models have been outlined, including a potential role for retroviral pathogens and directly cytopathic effects. © 2008 Elsevier Inc. All rights reserved.


Publication metadata

Author(s): Jones DEJ

Publication type: Article

Publication status: Published

Journal: Clinics in Liver Disease

Year: 2008

Volume: 12

Issue: 2

Pages: 305-321

ISSN (print): 1089-3261

ISSN (electronic): 1557-8224

Publisher: W.B. Saunders Co.

URL: http://dx.doi.org/10.1016/j.cld.2008.02.004

DOI: 10.1016/j.cld.2008.02.004

PubMed id: 18456182


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