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Disease Markers
Volume 29, Issue 6, Pages 329-336

Environmental Agents Involved in the Cause of Primary Biliary Cirrhosis

Elias Kouroumalis

Professor of Gastroenterology, University of Crete Medical School, Head, Department Of Gastroenterology, University Hospital, PO Box 1352, 71100 Heraklion, Crete, Greece

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 an immune mediated chronic cholestatic liver disease with a slowly progressive course It is a universal disease with a reported latitudinal gradient in prevalence and incidence. The aetiology of primary biliary cirrhosis is still unknown. It is characterized by a 60% concordance in monozygotic twins and is considered an autoimmune disease because of several features common to other autoimmune conditions and the relatively homogeneous serological and biochemical features. However geoepidemiological and clinical studies strongly imply that environmental factors also play an important role. It is accepted that the disease is clearly the result of a combination of genetic and environmental factors. Several risk factors have been suggested to be associated with PBC, including exposure to infectious agents and chemical xenobiotics. This review will attempt to place such factors in perspective.