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.
Highlights
The aetiology of primary biliary cirrhosis (PBC), an immune mediated and potentially fatal cholestatic liver disease, remains unsolved [1,2,3]
Infectious and environmental factors have been considered important for the development of the disease [4,5,6,7,8,9,10]
Immunological investigations in biological material obtained from patients at early stages of the disease, and studies in experimental models resembling the human disease have revealed the pathogenic potential of specific environmental agents [10,23,24]
Summary
The aetiology of primary biliary cirrhosis (PBC), an immune mediated and potentially fatal cholestatic liver disease, remains unsolved [1,2,3]. Infectious and environmental factors have been considered important for the development of the disease [4,5,6,7,8,9,10]. Immunological investigations in biological material obtained from patients at early stages of the disease, and studies in experimental models resembling the human disease have revealed the pathogenic potential of specific environmental agents [10,23,24]. D. Smyk et al / Environment and primary biliary cirrhosis cal, clinical and experimental studies investigating environmental factors which may contribute to the development of primary biliary cirrhosis
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