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Disease Markers
Volume 29 (2010), Issue 6, Pages 301-311

The Role of E. coli Infection in the Pathogenesis of Primary Biliary Cirrhosis

Dimitrios P. Bogdanos,1 Harold Baum,1 Diego Vergani,1 and Andrew K. Burroughs2

1Institute of Liver Studies, King's College London School of Medicine at King's College Hospital, Denmark Hill Campus, London, UK
2The Sheila Sherlock Liver Centre, and University Department of Surgery, Royal Free Hospital, London, UK

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.


Among various infectious agents possibly involved in the pathogenesis of primary biliary cirrhosis (PBC), Escherichia Coli (E. coli) has received special attention because of epidemiological and experimental evidence linking this bacterium with the disease's development. This review discusses early and more recent epidemiological studies associating recurrent urinary tract infections with E. coli and the development of PBC. We also critically review data provided over the years demonstrating disease-specific humoral and cellular immune responses against E. coli antigens in patients with PBC. Finally, we assess the relevance of experimental findings reporting cross-reactive immunity between mimicking sequences of E. coli and the major PBC mitochondrial antigens in the pathogenesis of the PBC. We also address the extent to which molecular mimicry and immunological cross-reactivity can be considered as a critical pathogenic process linking infection with self destruction.