Literature DB >> 31063540

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

Ivan Guerra1, Luis Bujanda2,3, Jesús Castro4, Olga Merino5, Joan Tosca6, Blau Camps7, Ana Gutiérrez3,8, Jordi Gordillo Ábalos9, Luisa de Castro10, Marisa Iborra3,11, Ana Y Carbajo12, Carlos Taxonera13, Iago Rodríguez-Lago14, Francisco Mesonero15, Ruth de Francisco16, Gonzalo J Gómez-Gómez17, María Chaparro3,18, Carlos A Tardillo19, Montserrat Rivero20, Alicia Algaba1, Eduardo Martín Arranz21, Fiorella Cañete3,22, Raquel Vicente23, Beatriz Sicilia24, Beatriz Antolín25, Vanessa Prieto26, Lucía Márquez27, José M Benítez28, Patricia Camo29, Marta Piqueras30, Carla J Gargallo31, Esther Hinojosa32, José M Huguet33, José L Pérez Calle34, Manuel Van Domselaar35, Cristina Rodriguez36, Xavier Calvet3,37, Carmen Muñoz-Villafranca38, Mariana F García-Sepulcre39, Patricia Munoz-Garrido2,3, Agnès Fernández-Clotet4, Laura Gómez Irwin5, Sherly Hernández6, Jordi Guardiola7,40, Laura Sempere8, Carlos González Muñoza9, Vicent Hernández10, Belén Beltrán3,11, Jesús Barrio12, Cristina Alba13, Irene Moraleja14, Antonio López-Sanromán15, Sabino Riestra16, Pilar Martínez Montiel17, Ana Garre3,18, Laura Arranz19, María José García20, María Dolores Martín Arranz21, Pilar Corsino23, Lara Arias24, Luis Fernández-Salazar25, Alejandra Fernández-Pordomingo26, Montserrat Andreu27, Eva Iglesias28, Yolanda Ber29, Raquel Mena30, María Teresa Arroyo Villarino31, María Mora32, Lucía Ruiz33, Pilar López-Serrano34, Isabel Blazquez35, Albert Villoria3,36, María Fernández37, Fernando Bermejo1, Jesus M Banales2,3,41, Eugeni Domènech3,22, Javier P Gisbert3,18.   

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.
Copyright © 2019 European Crohn’s and Colitis Organisation (ECCO). Published by Oxford University Press. All rights reserved. For permissions, please email: journals.permissions@oup.com.

Entities:  

Keywords:  Primary sclerosing cholangitis; inflammatory bowel disease; malignancy

Mesh:

Year:  2019        PMID: 31063540     DOI: 10.1093/ecco-jcc/jjz094

Source DB:  PubMed          Journal:  J Crohns Colitis        ISSN: 1873-9946            Impact factor:   9.071


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