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Issue title: Transient Environmental Agents Involved in the Cause of Primary Biliary Cirrhosis
Article type: Research Article
Authors: Smyk, Daniel | Mytilinaiou, Maria G. | Rigopoulou, Eirini I.; | Bogdanos, Dimitrios P.
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: dimitrios.bogdanos@kcl.ac.uk; 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.
Keywords: Autoimmunity, disease, bile ducts, cholestasis, liver, immunity, tolerance, mimicry, cross-reactivity, environment
DOI: 10.3233/DMA-2010-0744
Journal: Disease Markers, vol. 29, no. 6, pp. 337-344, 2010
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