Clinical Characteristics, Associated Malignancies and Management of Primary Sclerosing Cholangitis in Inflammatory Bowel Disease Patients: A Multicentre Retrospective Cohort Study
Authors: 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)
Published: May 2019

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.