Population-based epidemiology, malignancy risk, and outcome of primary sclerosing cholangitisстатья из журнала
Аннотация: 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.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.
Год издания: 2013
Авторы: Kirsten Boonstra, Rinse K. Weersma, Karel J. van Erpecum, Erik A. Rauws, B.W.M. Spanier, Alexander C. Poen, Karin M. van Nieuwkerk, Joost P.H. Drenth, Ben J.�M. Witteman, Hans A.R.E. Tuynman, A.H.J. Naber, Paul J. Kingma, Henk R. van Buuren, Bart van Hoek, Frank P. Vleggaar, Nan van Geloven, Ulrich Beuers, Cyriel Y. Ponsioen
Издательство: Lippincott Williams & Wilkins
Источник: Hepatology
Ключевые слова: Liver Diseases and Immunity, Liver Disease Diagnosis and Treatment, Gallbladder and Bile Duct Disorders
Другие ссылки: Hepatology (PDF)
Hepatology (HTML)
Leiden Repository (Leiden University) (PDF)
Leiden Repository (Leiden University) (HTML)
PubMed (HTML)
Hepatology (HTML)
Leiden Repository (Leiden University) (PDF)
Leiden Repository (Leiden University) (HTML)
PubMed (HTML)
Открытый доступ: bronze
Том: 58
Выпуск: 6
Страницы: 2045–2055