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

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 characte...

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Detalhes bibliográficos
Autores: Guerra, I, Bujanda, L, Castro, J, Merino, O, Tosca, J, Camps, B, GUTIERREZ, A., Abalos, JG, de Castro, L, Iborra, M, Carbajo, AY, Taxonera, C, Rodriguez-Lago, I, Mesonero, F, de Francisco, R, Gomez-Gomez, GJ, Chaparro, M, Tardillo, CA, Rivero, M, Algaba, A, Arranz, EM, Canete, F, Vicente, R, Sicilia, B, Antolin, B, Prieto, V, Marquez, L, Benitez, JM, Camo, P, Piqueras, M, Gargallo, CJ, Hinojosa, E, Huguet, JM, Calle, JLP, Van Domselaar, M, Rodriguez, C, Calvet, X, Munoz-Villafranca, C, Garcia-Sepulcre, MF, Munoz-Garrido, P, Fernandez-Clotet, A, Irwin, LG, Hernandez, S, Guardiola, J, SEMPERE, L., Munoza, CG, Hernandez, V, Beltran, B, Barrio, J, Alba, C, Moraleja, I, Lopez-Sanroman, A, Riestra, S, Montiel, PM, Garre, A, Arranz, L, Garcia, MJ, Arranz, MDM, Corsino, P, Arias, L, Fernandez-Salazar, L, Fernandez-Pordomingo, A, Andreu, M, Iglesias, E, Ber, Y, Mena, R, Villarino, MTA, Mora, M, Ruiz, L, Lopez-Serrano, P, Blazquez, I, Villoria, A, Fernandez, M, Bermejo, F, Banales, JM, Domenech, E, Gisbert, JP
Formato: artículo
Estado:Versión publicada
Fecha de publicación:2019
País:España
Recursos:Fundación para el Fomento de la Investigación Sanitaria y Biomédica de la Comunitat Valenciana (FISABIO)
Repositorio:r-FISABIO. Repositorio Institucional de Producción Científica
OAI Identifier:oai:fisabio.fundanetsuite.com:p5457
Acesso em linha:https://fisabio.portalinvestigacion.com/publicaciones/5457
Access Level:acceso abierto
Palavra-chave:Primary sclerosing cholangitis
inflammatory bowel disease
malignancy
Descrição
Resumo: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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