Clinical Characteristics, Associated Malignancies and Management of Primary Sclerosing Cholangitis in Inflammatory Bowel Disease Patients: A Multicentre Retrospective Cohort Study.
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Guerra Ivan, Bujanda Luis, Castro Jesús, Merino Olga, Tosca Joan, Camps Blau, Gutiérrez Ana, Gordillo Ábalos Jordi, de Castro Luisa, Iborra Marisa, Carbajo Ana Y, Taxonera Carlos, Rodríguez-Lago Iago, Mesonero Francisco, de Francisco Ruth, Gómez-Gómez Gonzalo J, Chaparro María, Tardillo Carlos A, Rivero Montserrat, Algaba Alicia, Martín Arranz Eduardo, Cañete Fiorella, Vicente Raquel, Sicilia Beatriz, Antolín Beatriz, Prieto Vanessa, Márquez Lucía, Benítez José M, Camo Patricia, Piqueras Marta, Gargallo Carla J, Hinojosa Esther, Huguet José M, Pérez Calle José L, Van Domselaar Manuel, Rodriguez Cristina, Calvet Xavier, Muñoz-Villafranca Carmen, García-Sepulcre Mariana F, Munoz-Garrido Patricia, Fernández-Clotet Agnès, Gómez Irwin Laura, Hernández Sherly, Guardiola Jordi, Sempere Laura, González Muñoza Carlos, Hernández Vicent, Beltrán Belén, Barrio Jesús, Alba Cristina, Moraleja Irene, López-Sanromán Antonio, Riestra Sabino, Martínez Montiel Pilar, Garre Ana, Arranz Laura, García María José, Martín Arranz María Dolores, Corsino Pilar, Arias Lara, Fernández-Salazar Luis, Fernández-Pordomingo Alejandra, Andreu Montserrat, Iglesias Eva, Ber Yolanda, Mena Raquel, Arroyo Villarino María Teresa, Mora María, Ruiz Lucía, López-Serrano Pilar, Blazquez Isabel, Villoria Albert, Fernández María, Bermejo Fernando, Banales Jesus M, Domènech Eugeni, Gisbert Javier P, Spanish GETECCU group (ENEIDA Project)
Аннотация
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.