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Primary biliary cirrhosis (PBC) and primary sclerosing cholangitis (PSC) are uncommon liver diseases of unknown etiology. Reported clustering of PBC cases may be due to environmental factors. Individuals with PBC have a high prevalence of thyroid disease and thyroid disease is reportedly more prevalent near Superfund toxic waste sites (SFS). The objective of this study was to examine the prevalence and potential clustering of individuals with PBC and PSC near SFS. De‐identified clinical and demographic data were used to determine the observed prevalence for each New York City zip code (n = 174) and borough (n = 5) of patients with PBC (PBC‐OLT) or PSC (PSC‐OLT) who were listed for liver transplantation. The expected prevalence was calculated using Organ Procurement and Transfer Network (OPTN) and U.S. Both PBC‐OLT patients and patients not listed for liver transplantation (PBC‐MSSM) were included in the cluster analysis.

Prevalence ratios of PBC‐OLT and PSC‐OLT cases were compared for each zip code and for each borough with regard to the proximity or density of SFS, respectively. SaTScan software was used to identify clusters of PBC‐OLT cases and PBC‐MSSM cases. Prevalence ratio of PBC‐OLT, not PSC‐OLT, was significantly higher in zip codes containing or adjacent to SFS (1.225 vs. El croquis mvrdv pdf viewer.

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0.670, respectively, P =.025). The borough of Staten Island had the highest prevalence ratio of PBC‐OLT cases and density of SFS. Significant clusters of both PBC‐OLT and PBC‐MSSM were identified surrounding SFS. In conclusion, toxin exposure may be a risk factor influencing the clustering of PBC cases. (H EPATOLOGY 2006;43:525–531.). Primary biliary cirrhosis (PBC) is a chronic, cholestatic liver disease of unclear etiology characterized by nonsuppurative intrahepatic portal tract inflammation and bile duct destruction, which may progress to cirrhosis.

Elevated serum IgM levels and high titer serum reactivity against mitochondrial autoantigens are the other hallmarks of the disease., Most individuals diagnosed with PBC, typically middle‐aged women, also suffer from other autoimmune diseases, such as autoimmune thyroid disease. The role of retroviral infection in the disease remains undefined and controversial., Large geographic variations in the prevalence of PBC have been reported. - Differences in both genetic susceptibility factors and exposure to environmental toxins are widely believed to contribute to this variability in the prevalence of PBC. Several studies support the importance of genetic susceptibility factors in the pathogenesis of PBC.

- Immune‐related gene polymorphisms are increased in frequency in PBC. Also, the concordance rate of PBC among monozygotic twins (60%) is significantly higher than among dizygotic twins (0%). The concordance rate in monozygotic twins is as high or higher than that observed in other autoimmune diseases believed to have a genetic component. Lastly, several studies have shown that the prevalence of PBC within families of affected individuals is significantly higher than in the general population., Differences in the local prevalence of PBC have been reported -,, and may reflect differences in exposure to environmental toxins. However, strong linkage to any specific environmental factor has not been identified except in a Japanese study showing an increased prevalence of PBC in Hiroshima among atomic bomb survivors.

Interestingly, a number of common chemical reagents, particularly aromatic and halogenated hydrocarbons, present in both industrial and household compounds may elicit mitochondrial autoantigen modifications in vitro that enhance autoantibody reactivity. - These chemical reagents are often the major toxins identified at toxic waste sites. In New York City, particularly hazardous toxic waste sites have been designated New York State Superfund sites (NYC SFS) by the Department of Environment and Conservation (DEC).

Deleterious effects on the immune system of individuals living near SFS have been reported by Vine et al., and thyroid disease was increased among women living near SFS in NYS containing polychlorinated biphenyls. Thus, we decided to determine if the prevalence of PBC is increased near NYC SFS employing data from several sources and multiple analytic methods. Abbreviations PBC, primary biliary cirrhosis; NYC SFS, New York City Superfund sites; DEC, Department of Environment and Conservation; PSC, primary sclerosing cholangitis; PBC‐OLT, PBC patients listed for liver transplantation; PSC‐OLT, PSC patients listed for liver transplantation; OPTN, Organ Procurement and Transfer Network; MSSM, Mount Sinai School of Medicine, New York; PBC‐MSSM, PBC patients followed at MSSM; spr, standardized prevalence ratio. Study Area and Data Used. New York City has a total area of 368 sq mi (953 km 2) consisting of 174 zip codes divided among 5 boroughs (Bronx, Brooklyn, Manhattan, Queens and Staten Island).