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Trends Immunol. 2009 Aug;30(8):415-20. doi: 10.1016/ Epub 2009 Jul 28.

The role of environmental factors in primary biliary cirrhosis.

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  • 1Department of Internal Medicine, IRCCS Istituto Clinico Humanitas, University of Milan, Rozzano, Milan 20089, Italy.


The etiology of primary biliary cirrhosis (PBC) is enigmatic, although it is clearly related to a combination of genetic predisposition and environmental stimulation. PBC is a chronic autoimmune cholestatic liver disease that occurs throughout the world with a reported latitudinal gradient in prevalence and incidence. PBC is also characterized by a 60% concordance in monozygotic twins and is considered a model autoimmune disease because of several features common to other conditions and the relatively homogeneous serological and biochemical features. 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.

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