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Disease Markers
Volume 29 (2010), Issue 6, Pages 277-286

Infectious Agents in the Pathogenesis of Primary Biliary Cirrhosis

Oscar-Danilo Ortega-Hernandez,1 Nancy-Agmon Levin,1,2 Arie Altman,1,2 and Yehuda Shoenfeld1,2

1The Zabludowicz Center for Autoimmune Diseases and Department of Medicine B, Sheba Medical Center, Tel-Aviv, Israel
2Tel-Aviv University, Tel-Aviv, Israel

Received 24 January 2011; Accepted 24 January 2011

Copyright © 2010 Hindawi Publishing Corporation. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.


Primary biliary cirrhosis (PBC) is a chronic progressive cholestatic liver disease which is characterized by the breakdown of self-tolerance to the highly conserved pyruvate dehydrogenase complex, specially the pyruvate dehydrogenase E2 complex (PDC-E2). The breakdown of the tolerance to such antigens leads to an autoimmune process characterized by portal inflammation and immune-mediated destruction of the intrahepatic bile ducts. Epidemiological studies have suggested that infections agents can trigger or even exacerbate the disease. Among other gram negative bacteria, Escherichia Coli, and Nosphingobium aromaticivorans are the most associated agents reported hitherto. Epidemiological and molecular evidence points towards molecular mimicry between some components of these microorganisms and specific amino-acid sequences that are present in proteins on normal cells of the biliary tract. In this review, we revisit all reports suggesting that infectious agents might be associated with the autoimmune pathogenesis of PBC. We also retrieve the immune molecular mimicry mechanisms that are likely involved with the autoimmune process in PBC.