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

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dc.contributor.author Weismueller, Tobias J.
dc.contributor.author Trivedi, Palak J.
dc.contributor.author Bergquist, Annika
dc.contributor.author Imam, Mohamad
dc.contributor.author Lenzen, Henrike
dc.contributor.author Ponsioen, Cyriel Y.
dc.contributor.author Holm, Kristian
dc.contributor.author Gotthardt, Daniel
dc.contributor.author Färkkilä, Martti
dc.contributor.author Marschall, Hanns-Ulrich
dc.contributor.author Thorburn, Douglas
dc.contributor.author Weersma, Rinse K.
dc.contributor.author Fevery, Johan
dc.contributor.author Mueller, Tobias
dc.contributor.author Chazouilleres, Olivier
dc.contributor.author Schulze, Kornelius
dc.contributor.author Lazaridis, Konstantinos N.
dc.contributor.author Almer, Sven
dc.contributor.author Pereira, Stephen P.
dc.contributor.author Levy, Cynthia
dc.contributor.author Mason, Andrew
dc.contributor.author Naess, Sigrid
dc.contributor.author Bowlus, Christopher L.
dc.contributor.author Floreani, Annarosa
dc.contributor.author Halilbasic, Emina
dc.contributor.author Yimam, Kidist K.
dc.contributor.author Milkiewicz, Piotr
dc.contributor.author Beuers, Ulrich
dc.contributor.author Huynh, Dep K.
dc.contributor.author Pares, Albert
dc.contributor.author Manser, Christine N.
dc.contributor.author Dalekos, George N.
dc.contributor.author Eksteen, Bertus
dc.contributor.author Invernizzi, Pietro
dc.contributor.author Berg, Christoph P.
dc.contributor.author Kirchner, Gabi I.
dc.contributor.author Sarrazin, Christoph
dc.contributor.author Zimmer, Vincent
dc.contributor.author Fabris, Luca
dc.contributor.author Braun, Felix
dc.contributor.author Marzioni, Marco
dc.contributor.author Juran, Brian D.
dc.contributor.author Said, Karouk
dc.contributor.author Rupp, Christian
dc.contributor.author Jokelainen, Kalle
dc.contributor.author de Valle, Maria Benito
dc.contributor.author Saffioti, Francesca
dc.contributor.author Cheung, Angela
dc.contributor.author Trauner, Michael
dc.contributor.author Schramm, Christoph
dc.contributor.author Int PSC Study Grp
dc.date.accessioned 2018-07-07T21:20:23Z
dc.date.available 2021-12-17T22:02:53Z
dc.date.issued 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-+ . https://doi.org/10.1053/j.gastro.2017.02.038
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.identifier.uri http://hdl.handle.net/10138/237011
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 DOSE URSODEOXYCHOLIC ACID
dc.subject PRIMARY BILIARY-CIRRHOSIS
dc.subject GENOME-WIDE ASSOCIATION
dc.subject POPULATION-BASED COHORT
dc.subject ULCERATIVE-COLITIS
dc.subject NATURAL-HISTORY
dc.subject RISK-FACTORS
dc.subject CLINICAL PRESENTATION
dc.subject CROHNS-DISEASE
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.doi https://doi.org/10.1053/j.gastro.2017.02.038
dc.relation.issn 0016-5085
dc.rights.accesslevel openAccess
dc.type.version publishedVersion

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