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J Crohns Colitis. 2019 May 7. pii: jjz094. doi: 10.1093/ecco-jcc/jjz094. [Epub ahead of print]

Clinical characteristics, associated malignancies and management of primary sclerosing cholangitis in inflammatory bowel disease patients: A multicenter retrospective cohort study.

Author information

1
Department of Gastroenterology of Hospital Universitario de Fuenlabrada and Instituto de Investigación del Hospital Universitario La Paz, IdiPaz, Madrid.
2
Department of Gastroenterology of Hospital Donostia/Instituto Biodonostia, Universidad del País Vasco UPV/EHU, Donostia-San Sebastián.
3
Department of Gastroenterology of Centro de Investigación Biomédica en Red de Enfermedades Hepáticas y Digestivas (CIBERehd), Spain.
4
Department of Gastroenterology of Hospital Clinic de Barcelona, Barcelona.
5
Department of Gastroenterology of Hospital Universitario de Cruces, Barakaldo, Vizcaya.
6
Department of Gastroenterology of Hospital Universitario Clínico de Valencia, Department of Medicine, University of Valencia, Valencia.
7
Department of Gastroenterology of Hospital Universitari de Bellvitge, Institut d'Investigació Biomèdica de Bellvitge (IDIBELL), l'Hospitalet de Llobregat.
8
Department of Gastroenterology of Hospital General Universitario de Alicante, Alicante.
9
Department of Gastroenterology of Hospital de la Santa Creu i Sant Pau, Barcelona.
10
Department of Gastroenterology of Complexo Hospitalario Universitario de Vigo, Instituto de Investigación Biomédica. Estructura Organizativa de Xestión Integrada de Vigo, Vigo.
11
Department of Gastroenterology of Hospital Universitario y Politécnico de La Fe, Valencia.
12
Department of Gastroenterology of Hospital Universitario Rio Hortega, Valladolid.
13
Department of Gastroenterology of Hospital Clínico San Carlos and Instituto de Investigación del Hospital Clínico San Carlos (IdISSC), Madrid.
14
Department of Gastroenterology of Hospital de Galdakao, Galdakao, Vizcaya.
15
Department of Gastroenterology of Hospital Universitario Ramón y Cajal, Madrid.
16
Department of Gastroenterology of Hospital Universitario Central De Asturias, Instituto de Investigación Sanitaria del Principado de Asturias (ISPA), Oviedo.
17
Departments of Gastroenterology of Hospital 12 de Octubre, Madrid.
18
Department of Gastroenterology of Hospital Universitario de La Princesa, Instituto de Investigación Sanitaria Princesa (IIS-IP), Universidad Autónoma de Madrid, Madrid.
19
Department of Gastroenterology of Hospital Universitario Nuestra Señora Candelaria, Santa Cruz De Tenerife.
20
Department of Gastroenterology of Hospital Universitario Marqués de Valdecilla and IDIVAL, Santander.
21
Department of Gastroenterology of Hospital Universitario La Paz and Instituto de Investigación del Hospital Universitario La Paz, IdiPaz, Madrid.
22
Department of Gastroenterology of Hospital Universitari Germans Trias i Pujol, Badalona.
23
Department of Gastroenterology of Hospital Universitario Miguel Servet, Zaragoza.
24
Department of Gastroenterology of Hospital Universitario de Burgos, Burgos.
25
Department of Gastroenterology of Hospital Clínico Universitario de Valladolid, Valladolid.
26
Department of Gastroenterology of Hospital Universitario de Salamanca, Salamanca.
27
Department of Gastroenterology of Parc de Salut Mar, Barcelona.
28
Department of Gastroenterology of Hospital Universitario Reina Sofía and Instituto Maimónides de Investigación Biomédica de Córdoba (IMIBIC), Córdoba.
29
Department of Gastroenterology of Hospital General San Jorge, Huesca.
30
Department of Gastroenterology of Consorci Sanitari Terrassa, Terrassa, Zaragoza.
31
Department of Gastroenterology of Hospital Clínico Universitario "Lozano Blesa" and Aragón Health Research Institute (IIS Aragón), Zaragoza.
32
Department of Gastroenterology of Hospital de Manises, Valencia.
33
Department of Gastroenterology of Hospital General Universitario de Valencia, Valencia.
34
Department of Gastroenterology of Hospital Universitario Fundación Alcorcón, Madrid.
35
Department of Gastroenterology of Hospital Universitario de Torrejón and Universidad Francisco de Vitoria, Madrid.
36
Department of Gastroenterology of Complejo hospitalario de Navarra, Pamplona.
37
Department of Gastroenterology of Corporació Sanitària Universitària Parc Taulí, Universitat Autònoma de Barcelona, Sabadell.
38
Department of Gastroenterology of Hospital Universitario de Basurto, Bilbao.
39
Department of Gastroenterology of Hospital General Universitario Elche, Alicante.
40
Department of Gastroenterology of Universitat de Barcelona.
41
Department of Gastroenterology of Ikerbasque (Basque Foundation for Sciencies), Bilbao, Spain.

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 AEG-REDCap electronic data capture tool.

RESULTS:

Two hundred seventy-seven 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, mean age at PSC diagnosis 40±16 years. Most patients (85.2%) were treated with ursodeoxycholic acid. Liver transplantation was required in 35 patients (12.6%) after 79 months (IQR 50-139). It was more common in intra- and extrahepatic PSC compared with small-duct PSC (16.3% vs. 3.3%; OR 5.7: 95%CI=1.7-19.3). The incidence rate of CRC since PSC diagnosis was 3.3 cases per 1,000 patient-years (95%CI=1.9-5.6). Having symptoms of PSC at PSC diagnosis was the only factor related with increased risk of CRC after IBD diagnosis (HR=3.3: 95%CI=1.1-9.9). CCA was detected in 7 patients (2.5%) with intra- and extrahepatic PSC, 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 and increased risk of CRC. CCA was only diagnosed in patients with intra- and extrahepatic PSC and was associated with a poor survival.

KEYWORDS:

inflammatory bowel disease; malignancy; primary sclerosing cholangitis

PMID:
31063540
DOI:
10.1093/ecco-jcc/jjz094

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