Patient Age, Sex, and Inflammatory Bowel Disease Phenotype Associate With Course of Primary Sclerosing Cholangitis

Show simple item record Weismueller, Tobias J. Trivedi, Palak J. Bergquist, Annika Imam, Mohamad Lenzen, Henrike Ponsioen, Cyriel Y. Holm, Kristian Gotthardt, Daniel Färkkilä, Martti Marschall, Hanns-Ulrich Thorburn, Douglas Weersma, Rinse K. Fevery, Johan Mueller, Tobias Chazouilleres, Olivier Schulze, Kornelius Lazaridis, Konstantinos N. Almer, Sven Pereira, Stephen P. Levy, Cynthia Mason, Andrew Naess, Sigrid Bowlus, Christopher L. Floreani, Annarosa Halilbasic, Emina Yimam, Kidist K. Milkiewicz, Piotr Beuers, Ulrich Huynh, Dep K. Pares, Albert Manser, Christine N. Dalekos, George N. Eksteen, Bertus Invernizzi, Pietro Berg, Christoph P. Kirchner, Gabi I. Sarrazin, Christoph Zimmer, Vincent Fabris, Luca Braun, Felix Marzioni, Marco Juran, Brian D. Said, Karouk Rupp, Christian Jokelainen, Kalle de Valle, Maria Benito Saffioti, Francesca Cheung, Angela Trauner, Michael Schramm, Christoph Int PSC Study Grp 2018-07-07T21:20:23Z 2021-12-17T22:02:53Z 2017-06
dc.identifier.citation Weismueller , T J , Trivedi , P J , Bergquist , A , Imam , M , Lenzen , H , Ponsioen , C Y , Holm , K , Gotthardt , D , Färkkilä , M , Marschall , H-U , Thorburn , D , Weersma , R K , Fevery , J , Mueller , T , Chazouilleres , O , Schulze , K , Lazaridis , K N , Almer , S , Pereira , S P , Levy , C , Mason , A , Naess , S , Bowlus , C L , Floreani , A , Halilbasic , E , Yimam , K K , Milkiewicz , P , Beuers , U , Huynh , D K , Pares , A , Manser , C N , Dalekos , G N , Eksteen , B , Invernizzi , P , Berg , C P , Kirchner , G I , Sarrazin , C , Zimmer , V , Fabris , L , Braun , F , Marzioni , M , Juran , B D , Said , K , Rupp , C , Jokelainen , K , de Valle , M B , Saffioti , F , Cheung , A , Trauner , M , Schramm , C & Int PSC Study Grp 2017 , ' Patient Age, Sex, and Inflammatory Bowel Disease Phenotype Associate With Course of Primary Sclerosing Cholangitis ' , Gastroenterology , vol. 152 , no. 8 , pp. 1975-+ .
dc.identifier.other PURE: 86600203
dc.identifier.other PURE UUID: 11352b9f-e76c-4a18-9859-8728d49ea5bd
dc.identifier.other WOS: 000402808600035
dc.identifier.other Scopus: 85020117784
dc.identifier.other ORCID: /0000-0002-0250-8559/work/39204260
dc.description.abstract BACKGROUND & AIMS: Primary sclerosing cholangitis (PSC) is an orphan hepatobiliary disorder associated with inflammatory bowel disease (IBD). We aimed to estimate the risk of disease progression based on distinct clinical phenotypes in a large international cohort of patients with PSC. METHODS: We performed a retrospective outcome analysis of patients diagnosed with PSC from 1980 through 2010 at 37 centers in Europe, North America, and Australia. For each patient, we collected data on sex, clinician-reported age at and date of PSC and IBD diagnoses, phenotypes of IBD and PSC, and date and indication of IBD-related surgeries. The primary and secondary endpoints were liver transplantation or death (LTD) and hepatopancreatobiliary malignancy, respectively. Cox proportional hazards models were applied to determine the effects of individual covariates on rates of clinical events, with time-to-event analysis ascertained through Kaplan-Meier estimates. RESULTS: Of the 7121 patients in the cohort, 2616 met the primary endpoint (median time to event of 14.5 years) and 721 developed hepatopancreatobiliary malignancy. The most common malignancy was cholangiocarcinoma (n = 594); patients of advanced age at diagnosis had an increased incidence compared with younger patients (incidence rate: 1.2 per 100 patient-years for patients younger than 20 years old, 6.0 per 100 patient-years for patients 21-30 years old, 9.0 per 100 patient-years for patients 31-40 years old, 14.0 per 100 patient-years for patients 4150 years old, 15.2 per 100 patient-years for patients 51-60 years old, and 21.0 per 100 patient-years for patients older than 60 years). Of all patients with PSC studied, 65.5% were men, 89.8% had classical or large-duct disease, and 70.0% developed IBD at some point. Assessing the development of IBD as a time-dependent covariate, Crohn's disease and no IBD (both vs ulcerative colitis) were associated with a lower risk of LTD (unadjusted hazard ratio [HR], 0.62; P en
dc.format.extent 18
dc.language.iso eng
dc.relation.ispartof Gastroenterology
dc.rights.uri info:eu-repo/semantics/openAccess
dc.subject Risk Stratification
dc.subject Immune-Mediated Liver Disease
dc.subject Autoimmune Liver Disease
dc.subject Cholestasis
dc.subject RISK-FACTORS
dc.subject SINGLE-CENTER
dc.subject 3121 General medicine, internal medicine and other clinical medicine
dc.title Patient Age, Sex, and Inflammatory Bowel Disease Phenotype Associate With Course of Primary Sclerosing Cholangitis en
dc.type Article
dc.contributor.organization Clinicum
dc.contributor.organization Department of Medicine
dc.contributor.organization Gastroenterologian yksikkö
dc.contributor.organization HUS Abdominal Center
dc.description.reviewstatus Peer reviewed
dc.relation.issn 0016-5085
dc.rights.accesslevel openAccess
dc.type.version publishedVersion

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