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Population‐based epidemiology, malignancy risk, and outcome of primary sclerosing cholangitis
Author(s) -
Boonstra Kirsten,
Weersma Rinse K.,
Erpecum Karel J.,
Rauws Erik A.,
Spanier B.W. Marcel,
Poen Alexander C.,
Nieuwkerk Karin M.,
Drenth Joost P.,
Witteman Ben J.,
Tuynman Hans A.,
Naber Anton H.,
Kingma Paul J.,
Buuren Henk R.,
Hoek Bart,
Vleggaar Frank P.,
Geloven Nan,
Beuers Ulrich,
Ponsioen Cyriel Y.
Publication year - 2013
Publication title -
hepatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 5.488
H-Index - 361
eISSN - 1527-3350
pISSN - 0270-9139
DOI - 10.1002/hep.26565
Subject(s) - primary sclerosing cholangitis , medicine , incidence (geometry) , liver transplantation , epidemiology , population , cohort , malignancy , ulcerative colitis , inflammatory bowel disease , cohort study , rochester epidemiology project , liver disease , transplantation , disease , population based study , environmental health , physics , optics
Extensive population‐based studies are much needed to accurately establish epidemiology and disease course in patients with primary sclerosing cholangitis (PSC). We aimed to obtain population‐based prevalence and incidence figures, insight in disease course with regard to survival, liver transplantation (LT), and occurrence of malignancies, as well as risk factors thereof. Four independent hospital databases were searched in 44 hospitals in a large geographically defined area of the Netherlands, comprising 50% of the population. In addition, all PSC patients in the three Dutch liver transplant centers and all inflammatory bowel disease (IBD) patients in the adherence area of a large district hospital were identified. All medical records were reviewed on‐site, verifying diagnosis. Five hundred and ninety PSC patients were identified, resulting in an incidence of 0.5 and a point prevalence of 6.0 per 100,000. Median follow up was 92 months. Estimated median survival from diagnosis until LT or PSC‐related death in the entire cohort was 21.3 years, as opposed to 13.2 years in the combined transplant centers cohort (n = 422; P < 0.0001). Colorectal carcinoma (CRC) risk was 10‐fold increased, as compared to ulcerative colitis controls, and developed at a much younger age (39 years; range, 26‐64), compared to IBD controls (59 years; range, 34‐73; P = 0.019). Colonoscopic surveillance was associated with significantly better outcome. Conclusion : This study exemplifies that, for relatively rare diseases, it is paramount to collect observational data from large, population‐based cohorts, because incidence and prevalence rates of PSC are markedly lower and survival much longer than previously reported. The selection of a bias‐free, population‐based cohort showed a significantly longer survival, compared to the tertiary referral cohort. CRC can develop at an early age, warranting surveillance from time of PSC diagnosis. (H epatology 2013; 58:2045–2055)