1. Clinical Characteristics, Associated Malignancies and Management of Primary Sclerosing Cholangitis in Inflammatory Bowel Disease Patients: A Multicentre Retrospective Cohort Study
- Author
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R. Vicente, María Teresa Arroyo Villarino, E Hinojosa, Ana Garre, Antonio López-Sanromán, Jesús Barrio, Ana Gutiérrez, Ana Yaiza Carbajo, Marisa Iborra, Iago Rodríguez-Lago, Joan Tosca, Luis Fernández-Salazar, Cristina Alba, Luísa Castro, Sabino Riestra, Alejandra Fernández-Pordomingo, Xavier Calvet, Albert Villoria, Pilar Martínez Montiel, Carla J. Gargallo, Carlos González Muñoza, M Piqueras, Yolanda Ber, Carlos Taxonera, Laura Arranz, Olga Merino, Beatriz Antolín, M F García-Sepulcre, Eugeni Domènech, B. Beltrán, Agnès Fernández-Clotet, Iván Guerra, Alicia Algaba, Jesus M. Banales, María Mora, Beatriz Sicilia, Raquel Mena, Cristina Rodríguez, Francisco Mesonero, Manuel Van Domselaar, Laura Gómez Irwin, Jordi Gordillo Ábalos, Irene Moraleja, Jordi Guardiola, Lucía Ruiz, María Chaparro, José María Huguet, Montserrat Rivero, Montserrat Andreu, Laura Sempere, María Rosario Fernández, María Dolores Martín Arranz, Luis Bujanda, Fiorella Cañete, María José García, Lara Arias, Eduardo Arranz, Fernando Bermejo, Gonzalo J Gómez-Gómez, Pilar López-Serrano, Patricia Camo, Jesús Castro, José Manuel Benítez, Blau Camps, Pilar Corsino, Sherly Hernández, Vicent Hernandez, Patricia Munoz-Garrido, Carmen Muñoz-Villafranca, Ruth de Francisco, Eva Iglesias, Javier P. Gisbert, C Tardillo, Isabel Blazquez, Vanessa Prieto, José Lázaro Pérez Calle, and Lucía Márquez
- Subjects
Adult ,Male ,medicine.medical_specialty ,endocrine system diseases ,Colorectal cancer ,Cholangitis, Sclerosing ,Risk Assessment ,Gastroenterology ,Inflammatory bowel disease ,digestive system ,Primary sclerosing cholangitis ,Cholangiocarcinoma ,Bile Ducts, Extrahepatic ,Risk Factors ,Interquartile range ,inflammatory bowel disease ,Internal medicine ,Humans ,Medicine ,Retrospective Studies ,business.industry ,Hazard ratio ,digestive, oral, and skin physiology ,Retrospective cohort study ,General Medicine ,Odds ratio ,Middle Aged ,Inflammatory Bowel Diseases ,medicine.disease ,Survival Analysis ,Ulcerative colitis ,digestive system diseases ,Patient Care Management ,Bile Ducts, Intrahepatic ,Spain ,Female ,Primary sclerosing cholangitis, inflammatory bowel disease, malignancy ,Colorectal Neoplasms ,business ,malignancy - 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.
- Published
- 2019