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Observational Study
. 2019 Dec 10;13(12):1492-1500.
doi: 10.1093/ecco-jcc/jjz094.

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

Ivan Guerra  1 , Luis Bujanda  2   3 , Jesús Castro  4 , Olga Merino  5 , Joan Tosca  6 , Blau Camps  7 , Ana Gutiérrez  3   8 , Jordi Gordillo Ábalos  9 , Luisa de Castro  10 , Marisa Iborra  3   11 , Ana Y Carbajo  12 , Carlos Taxonera  13 , Iago Rodríguez-Lago  14 , Francisco Mesonero  15 , Ruth de Francisco  16 , Gonzalo J Gómez-Gómez  17 , María Chaparro  3   18 , Carlos A Tardillo  19 , Montserrat Rivero  20 , Alicia Algaba  1 , Eduardo Martín Arranz  21 , Fiorella Cañete  3   22 , Raquel Vicente  23 , Beatriz Sicilia  24 , Beatriz Antolín  25 , Vanessa Prieto  26 , Lucía Márquez  27 , José M Benítez  28 , Patricia Camo  29 , Marta Piqueras  30 , Carla J Gargallo  31 , Esther Hinojosa  32 , José M Huguet  33 , José L Pérez Calle  34 , Manuel Van Domselaar  35 , Cristina Rodriguez  36 , Xavier Calvet  3   37 , Carmen Muñoz-Villafranca  38 , Mariana F García-Sepulcre  39 , Patricia Munoz-Garrido  2   3 , Agnès Fernández-Clotet  4 , Laura Gómez Irwin  5 , Sherly Hernández  6 , Jordi Guardiola  7   40 , Laura Sempere  8 , Carlos González Muñoza  9 , Vicent Hernández  10 , Belén Beltrán  3   11 , Jesús Barrio  12 , Cristina Alba  13 , Irene Moraleja  14 , Antonio López-Sanromán  15 , Sabino Riestra  16 , Pilar Martínez Montiel  17 , Ana Garre  3   18 , Laura Arranz  19 , María José García  20 , María Dolores Martín Arranz  21 , Pilar Corsino  23 , Lara Arias  24 , Luis Fernández-Salazar  25 , Alejandra Fernández-Pordomingo  26 , Montserrat Andreu  27 , Eva Iglesias  28 , Yolanda Ber  29 , Raquel Mena  30 , María Teresa Arroyo Villarino  31 , María Mora  32 , Lucía Ruiz  33 , Pilar López-Serrano  34 , Isabel Blazquez  35 , Albert Villoria  3   36 , María Fernández  37 , Fernando Bermejo  1 , Jesus M Banales  2   3   41 , Eugeni Domènech  3   22 , Javier P Gisbert  3   18 ; Spanish GETECCU group (ENEIDA Project)
Affiliations
Observational Study

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

Ivan Guerra et al. J Crohns Colitis. .

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.

Keywords: Primary sclerosing cholangitis; inflammatory bowel disease; malignancy.

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