Affiliations: Department of Liver Diseases, Mount Sinai Medical
Center, New York, NY, USA
Note: [] Corresponding author: Nancy Bach, MD, Division of Liver
Diseases/ Transplant Institute, Mount Sinai Medical Center, Box 1101, 5 East
98th Street, New York, New York 10029, USA. Tel.: +1 212 241 0034; E-mail:
nancy.bach@mountsinai.org
Abstract: Primary biliary cirrhosis (PBC) is an autoimmune disease of unclear
etiology. It is a chronic, progressive condition that causes intrahepatic
ductal destruction ultimately leading to symptoms of cholestasis, cirrhosis and
liver failure. The disease predominantly affects middle aged Caucasian women.
It has a predilection to certain regions and is found in higher incidences in
North America and Northern Europe. It also has a genetic predisposition with a
concordance rate of 60% among monozygotic twins. Combinations of genetic and environmental factors
are proposed in the pathogenesis of this disease with a compelling body of
evidence that suggests a role for both these factors. This review will
elucidate data on the proposed environmental agents involved the disease's
pathogenesis including xenobiotic and microbial exposure and present some of
the supporting epidemiologic data.
