Affiliations: Institute of Liver Studies, King's College London
School of Medicine at King's College Hospital, Denmark Hill Campus, London,
UK | Department of Medicine and Research Laboratory of
Internal Medicine, Medical School, University of Thessaly, Larissa, Thessaly,
Greece | Institute of Biomedical Research and Technology,
Centre for Research and Technology-Thessaly (CE.RE.TE.TH), Larissa, Thessaly,
Greece
Note: [] Corresponding author: Dimitrios P. Bogdanos, MD, PhD, Clinical
Senior Lecturer/Consultant, Liver Immunopathology & Liver
Immunodiagnostics, Institute of Liver Studies, King's College London Medical
School at King's College Hospital, Denmark Hill Campus, London, SE5 9RS, UK.
Tel./Fax: +44 2032993397; E-mail: [email protected];
http://www.bogdanoslab.com
Abstract: Various environmental factors have been proposed as triggers of
primary biliary cirrhosis (PBC), a progressive autoimmune cholestatic liver
disease which is characterised by the destruction of the small intrahepatic
bile ducts. Support for their pathogenic role in PBC is provided by
epidemiological studies reporting familial clustering and clusters of the
disease within a given geographical area. The seminal study by Triger reporting
that the great majority of PBC cases in the English city of Sheffield drank
water from a specific water reservoir, has been followed by studies reporting
disease 'hot spots' within a restricted geographic region of the former coal
mining area of Newcastle. The New York study reporting an increased risk and
significant clustering of PBC cases near toxic federal waste disposal sites has
added strength to the notion that environmental factors, possibly in the form
of infectious agents or toxic/chemical environmental factors in areas of
contaminated land, water or polluted air may play a key role in the development
of the disease. This review discusses the findings of reports investigating
environmental factors which may contribute to the cause of primary biliary
cirrhosis.
