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Title: Clinical Characteristics, Associated Malignancies and Management of Primary Sclerosing Cholangitis in Inflammatory Bowel Disease Patients: A Multicentre Retrospective Cohort Study.
Author: Guerra I, Bujanda L, Castro J, Merino O, Tosca J, Camps B, Gutiérrez A, Gordillo Ábalos J, de Castro L, Iborra M, Carbajo AY, Taxonera C, Rodríguez-Lago I, Mesonero F, de Francisco R, Gómez-Gómez GJ, Chaparro M, Tardillo CA, Rivero M, Algaba A, Martín Arranz E, Cañete F, Vicente R, Sicilia B, Antolín B, Prieto V, Márquez L, Benítez JM, Camo P, Piqueras M, Gargallo CJ, Hinojosa E, Huguet JM, Pérez Calle JL, Van Domselaar M, Rodriguez C, Calvet X, Muñoz-Villafranca C, García-Sepulcre MF, Munoz-Garrido P, Fernández-Clotet A, Gómez Irwin L, Hernández S, Guardiola J, Sempere L, González Muñoza C, Hernández V, Beltrán B, Barrio J, Alba C, Moraleja I, López-Sanromán A, Riestra S, Martínez Montiel P, Garre A, Arranz L, García MJ, Martín Arranz MD, Corsino P, Arias L, Fernández-Salazar L, Fernández-Pordomingo A, Andreu M, Iglesias E, Ber Y, Mena R, Arroyo Villarino MT, Mora M, Ruiz L, López-Serrano P, Blazquez I, Villoria A, Fernández M, Bermejo F, Banales JM, Domènech E, Gisbert JP, Spanish GETECCU group (ENEIDA Project).
Journal: J Crohns Colitis; 2019 Dec 10; 13(12):1492-1500. PubMed ID: 31063540.
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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