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RESEARCH PRODUCT

Clinical Characteristics, Associated Malignancies and Management of Primary Sclerosing Cholangitis in Inflammatory Bowel Disease Patients: A Multicentre Retrospective Cohort Study

R. VicenteMaría Teresa Arroyo VillarinoE HinojosaAna GarreAntonio López-sanrománJesús BarrioAna GutiérrezAna Yaiza CarbajoMarisa IborraIago Rodríguez-lagoJoan ToscaLuis Fernández-salazarCristina AlbaLuísa CastroSabino RiestraAlejandra Fernández-pordomingoXavier CalvetAlbert VilloriaPilar Martínez MontielCarla J. GargalloCarlos González MuñozaM PiquerasYolanda BerCarlos TaxoneraLaura ArranzOlga MerinoBeatriz AntolínM F García-sepulcreEugeni DomènechB. BeltránAgnès Fernández-clotetIván GuerraAlicia AlgabaJesus M. BanalesJesus M. BanalesMaría MoraBeatriz SiciliaRaquel MenaCristina RodríguezFrancisco MesoneroManuel Van DomselaarLaura Gómez IrwinJordi Gordillo ÁBalosIrene MoralejaJordi GuardiolaLucía RuizMaría ChaparroJosé María HuguetMontserrat RiveroMontserrat AndreuLaura SempereMaría Rosario FernándezMaría Dolores Martín ArranzLuis BujandaFiorella CañeteMaría José GarcíaLara AriasEduardo ArranzFernando BermejoGonzalo J Gómez-gómezPilar López-serranoPatricia CamoJesús CastroJosé Manuel BenítezBlau CampsPilar CorsinoSherly HernándezVicent HernandezPatricia Munoz-garridoCarmen Muñoz-villafrancaRuth De FranciscoEva IglesiasJavier P. GisbertC TardilloIsabel BlazquezVanessa PrietoJosé Lázaro Pérez CalleLucía Márquez

subject

AdultMalemedicine.medical_specialtyendocrine system diseasesColorectal cancerCholangitis SclerosingRisk AssessmentGastroenterologyInflammatory bowel diseasedigestive systemPrimary sclerosing cholangitisCholangiocarcinomaBile Ducts ExtrahepaticRisk FactorsInterquartile rangeinflammatory bowel diseaseInternal medicineHumansMedicineRetrospective Studiesbusiness.industryPrimary sclerosing cholangitisHazard ratiodigestive oral and skin physiologyGastroenterologyRetrospective cohort studyGeneral MedicineOdds ratioMiddle AgedInflammatory Bowel Diseasesmedicine.diseaseSurvival AnalysisUlcerative colitisdigestive system diseasesPatient Care ManagementBile Ducts IntrahepaticSpainFemalePrimary sclerosing cholangitis inflammatory bowel disease malignancyColorectal Neoplasmsbusinessmalignancy

description

Abstract Background and Aims Primary sclerosing cholangitis [PSC] is usually associated with inflammatory bowel disease [IBD]. An increased risk of malignancies, mainly colorectal cancer [CRC] and cholangiocarcinoma [CCA], has been reported in PSC-IBD patients. Our aim was to determine the clinical characteristics and management of PSC in IBD patients, and the factors associated with malignancies. Methods PSC-IBD patients were identified from the Spanish ENEIDA registry of GETECCU. Additional data were collected using the AEG-REDCap electronic data capture tool. Results In total, 277 PSC-IBD patients were included, with an incidence rate of 61 PSC cases per 100 000 IBD patient-years, 69.7% men, 67.5% ulcerative colitis and mean age at PSC diagnosis of 40 ± 16 years. Most patients [85.2%] were treated with ursodeoxycholic acid. Liver transplantation was required in 35 patients [12.6%] after 79 months (interquartile range [IQR] 50–139). It was more common in intra- and extrahepatic PSC compared with small-duct PSC (16.3% vs 3.3%; odds ratio [OR] 5.7: 95% confidence interval [CI] = 1.7–19.3). The incidence rate of CRC since PSC diagnosis was 3.3 cases per 1000 patient-years [95% CI = 1.9–5.6]. Having symptoms of PSC at PSC diagnosis was the only factor related to an increased risk of CRC after IBD diagnosis [hazard ratio= 3.3: 95% CI = 1.1–9.9]. CCA was detected in seven patients [2.5%] with intra- and extrahepatic PSC, with median age of 42 years [IQR 39–53], and presented a lower life expectancy compared with patients without CCA and patients with or without CRC. Conclusions PSC-IBD patients with symptoms of PSC at PSC diagnosis have an increased risk of CRC. CCA was only diagnosed in patients with intra- and extrahepatic PSC and was associated with poor survival.

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