Abstract
Primary biliary cirrhosis (PBC) is an immune mediated chronic cholestatic liver disease with a slowly progressive course It is a universal disease with a reported latitudinal gradient in prevalence and incidence. The aetiology of primary biliary cirrhosis is still unknown. It is characterized by a 60% concordance in monozygotic twins and is considered an autoimmune disease because of several features common to other autoimmune conditions and the relatively homogeneous serological and biochemical features. However geoepidemiological and clinical studies strongly imply that environmental factors also play an important role. It is accepted that the disease is clearly the result of a combination of genetic and environmental factors. 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.
Highlights
Several risk factors have been suggested to be associated with Primary biliary cirrhosis (PBC), including exposure to infectious agents and chemical xenobiotics
Primary biliary cirrhosis (PBC) is a chronic inflammatory autoimmune disease that mainly targets the cholangiocytes of the interlobular bile ducts in the liver [1]
Genetic factors likely play an important role in conferring susceptibility to PBC, as indicated by the high concordance rate among monozygotic twins [7], the highest among autoimmune diseases and the increased incidence among first-degree relatives of affected individuals [8]
Summary
Primary biliary cirrhosis (PBC) is a chronic inflammatory autoimmune disease that mainly targets the cholangiocytes of the interlobular bile ducts in the liver [1]. Space – time clustering is distinct from spatial clustering and is said to occur when an excess of cases is observed within small geographical areas over limited temporal periods, and this pattern cannot be explained by general excesses in those areas or at those periods [13] Another recent study from New York examined the prevalence of PBC and PSC near superfund areas and reported significant clusters of PBC surrounding toxic sites [14]. In synopsis, these observations gave rise to the hypothesis of a chemical environmental factor, potentially associated with contaminated land, which could either trigger disease or cause disease through a direct toxic effect [15].
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