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      The prevalence, incidence and natural history of primary sclerosing cholangitis in an ethnically diverse population

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          Abstract

          Background

          Primary sclerosing cholangitis (PSC) is a rare chronic cholestatic liver disease often associated with inflammatory bowel diseases (IBD). Current epidemiological data are limited to studies of predominantly Caucasian populations. Our aim was to define the epidemiology of PSC in a large, ethnically diverse US population.

          Methods

          The Northern California Kaiser Permanente (KP) database includes records from over 3 million people and was searched for cases of PSC between January 2000 and October 2006. All identified charts were reviewed for diagnosis confirmation, IBD co-morbidity, and major natural history endpoints.

          Results

          We identified 169 (101 males) cases fulfilling PSC diagnostic criteria with a mean age at diagnosis of 44 years (range 11-81). The age-adjusted point prevalence was 4.15 per 100,000 on December 31, 2005. The age-adjusted incidence per 100,000 person-years was not significantly greater in men 0.45 (95% CI 0.33 - 0.61) than women 0.37 (95% CI 0.26 - 0.51). IBD was present in 109/169 (64.5%) cases and was significantly more frequent in men than women with PSC (73.3% and 51.5%, respectively, p = 0.005). The cumulative average yearly mortality rate was 1.9%. Age and serum sodium, creatinine and bilirubin at diagnosis and albumin at last entry were identified as significant factors associated with death, liver transplant or cholangiocarcinoma.

          Conclusions

          The incidence and prevalence of PSC observed in a representative Northern California population are lower compared to previous studies in Caucasian populations and this might reflect differences in the incidence of PSC among various ethnic groups.

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          Most cited references35

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          PSC-IBD: a unique form of inflammatory bowel disease associated with primary sclerosing cholangitis.

          Inflammatory bowel disease associated with primary sclerosing cholangitis (PSC-IBD) may have a high prevalence of rectal sparing, backwash ileitis, and colorectal neoplasia. To describe the clinical features and outcomes of PSC-IBD and compare these to a group of chronic ulcerative colitis (CUC) patients. The medical records of all patients with PSC-IBD evaluated at the Mayo Clinic Rochester between 1987 and 1992 were abstracted for information on endoscopic and histological features, colorectal neoplasia, surgery, and other clinical outcomes. Patients referred for colorectal neoplasia and those who did not undergo colonoscopy with biopsies were excluded. A control group of CUC patients matched for sex, duration of IBD at first clinic visit, and calendar year of first clinic visit was identified, and similar information was abstracted. Seventy one PSC-IBD patients and 142 CUC patients without PSC were identified. Rectal sparing and backwash ileitis were more common in the PSC-IBD group (52% and 51%, respectively) than in controls (6% and 7%, respectively). Overall, colorectal neoplasia developed in 18 cases and 15 controls, including 11 cancers (seven cases and four controls). An increased risk of colorectal neoplasia or death was not detected in a matched analysis. Although the cumulative incidence of colorectal neoplasia was higher in cases (33%) than in controls (13%) at five years, this was of borderline statistical significance (p=0.054, unmatched log rank test). Overall survival from first clinic visit was significantly worse among cases (79% v 97%) at five years (p<0.001, unmatched log rank test). PSC-IBD is frequently characterised by rectal sparing and backwash ileitis. Colorectal neoplasia develops in a substantial fraction and overall survival is worse. PSC-IBD may represent a distinct IBD phenotype.
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            Incidence, clinical spectrum, and outcomes of primary sclerosing cholangitis in a United States community.

            The epidemiology of primary sclerosing cholangitis (PSC) in the United States is unknown. We report the incidence, clinical spectrum, and outcomes of PSC in Olmsted County, Minnesota. Using the Rochester Epidemiology Project, a medical records linkage system in Olmsted County, Minnesota, we identified county residents with PSC, and the diagnosis was confirmed according to clinical, biochemical, radiographic, and histologic criteria. Twenty-two patients met diagnostic criteria for PSC in 1976-2000. The age-adjusted (to 2000 U.S. whites) incidence of PSC in men was 1.25 per 100,000 person-years (95% CI, 0.70 to 2.06) compared with 0.54 per 100,000 person-years (95% CI, 0.22 to 1.12) in women. The prevalence of PSC in 2000 was 20.9 per 100,000 men (95% CI, 9.5 to 32.4) and only 6.3 per 100,000 women (95% CI, 0.1 to 12.5). Seventy-three percent of cases had inflammatory bowel disease, the majority with ulcerative colitis. Survival among PSC patients was significantly less than expected for the Minnesota white population of similar age and gender (P < 0.001). These data represent the first population-based estimates of the incidence and prevalence of PSC in the United States. The incidence and prevalence of PSC were approximately one third of those previously described for primary biliary cirrhosis in the same population. Our data suggest that the prevalence of PSC in the United States, with its attendant medical burdens, is significantly greater than previously estimated.
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              Confidence intervals for weighted sums of Poisson parameters.

              Directly standardized mortality rates are examples of weighted sums of Poisson rate parameters. If the numbers of events are large then normal approximations can be used to calculate confidence intervals, but these are inadequate if the numbers are small. We present a method for obtaining approximate confidence limits for the weighted sum of Poisson parameters as linear functions of the confidence limits for a single Poisson parameter, the unweighted sum. The location and length of the proposed interval depend on the method used to obtain confidence limits for the single parameter. Therefore several methods for obtaining confidence intervals for a single Poisson parameter are compared. For single parameters and for weighted sums of parameters, simulation suggests that the coverage of the proposed intervals is close to the nominal confidence levels. The method is illustrated using data on rates of myocardial infarction obtained as part of the WHO MONICA Project in Augsburg, Federal Republic of Germany.
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                Author and article information

                Journal
                BMC Gastroenterol
                BMC Gastroenterology
                BioMed Central
                1471-230X
                2011
                18 July 2011
                : 11
                : 83
                Affiliations
                [1 ]Department of Medicine, University of California Davis Medical Center, Sacramento, CA USA
                [2 ]Division of Gastroenterology, Kaiser Permanente Medical Group, Sacramento, CA USA
                [3 ]Autoimmunity and Metabolism Unit, IRCCS Istituto Clinico Humanitas, Rozzano, Milan, Italy
                [4 ]Division of Rheumatology, Allergy and Clinical Immunology, University of California Davis, Davis, CA USA
                [5 ]Department of Public Health Sciences, Division of Biostatistics, University of California Davis, Davis, CA USA
                [6 ]Division of Gastroenterology and Hepatology, University of California Davis Medical Center, Sacramento, CA USA
                Article
                1471-230X-11-83
                10.1186/1471-230X-11-83
                3160402
                21767410
                560dcf79-c9bf-4d8f-b81c-2adcdaa91993
                Copyright ©2011 Toy et al; licensee BioMed Central Ltd.

                This is an Open Access article distributed under the terms of the Creative Commons Attribution License ( http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

                History
                : 19 April 2011
                : 18 July 2011
                Categories
                Research Article

                Gastroenterology & Hepatology
                inflammatory bowel disease,cholangiocarcinoma,epidemiology,transplantation

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