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

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 characte...

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Autores: Guerra, I, Bujanda, L, Castro, J, Merino, O, Tosca, J, Camps, B, GUTIERREZ, A., Abalos, JG, de Castro, L, Iborra, M, Carbajo, AY, Taxonera, C, Rodriguez-Lago, I, Mesonero, F, de Francisco, R, Gomez-Gomez, GJ, Chaparro, M, Tardillo, CA, Rivero, M, Algaba, A, Arranz, EM, Canete, F, Vicente, R, Sicilia, B, Antolin, B, Prieto, V, Marquez, L, Benitez, JM, Camo, P, Piqueras, M, Gargallo, CJ, Hinojosa, E, Huguet, JM, Calle, JLP, Van Domselaar, M, Rodriguez, C, Calvet, X, Munoz-Villafranca, C, Garcia-Sepulcre, MF, Munoz-Garrido, P, Fernandez-Clotet, A, Irwin, LG, Hernandez, S, Guardiola, J, SEMPERE, L., Munoza, CG, Hernandez, V, Beltran, B, Barrio, J, Alba, C, Moraleja, I, Lopez-Sanroman, A, Riestra, S, Montiel, PM, Garre, A, Arranz, L, Garcia, MJ, Arranz, MDM, Corsino, P, Arias, L, Fernandez-Salazar, L, Fernandez-Pordomingo, A, Andreu, M, Iglesias, E, Ber, Y, Mena, R, Villarino, MTA, Mora, M, Ruiz, L, Lopez-Serrano, P, Blazquez, I, Villoria, A, Fernandez, M, Bermejo, F, Banales, JM, Domenech, E, Gisbert, JP
Tipo de recurso: artículo
Estado:Versión publicada
Fecha de publicación:2019
País:España
Institución:Fundación para el Fomento de la Investigación Sanitaria y Biomédica de la Comunitat Valenciana (FISABIO)
Repositorio:r-FISABIO. Repositorio Institucional de Producción Científica
OAI Identifier:oai:fisabio.fundanetsuite.com:p5457
Acceso en línea:https://fisabio.portalinvestigacion.com/publicaciones/5457
Access Level:acceso abierto
Palabra clave:Primary sclerosing cholangitis
inflammatory bowel disease
malignancy
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spelling Clinical Characteristics, Associated Malignancies and Management of Primary Sclerosing Cholangitis in Inflammatory Bowel Disease Patients: A Multicentre Retrospective Cohort StudyGuerra, IBujanda, LCastro, JMerino, OTosca, JCamps, BGUTIERREZ, A.Abalos, JGde Castro, LIborra, MCarbajo, AYTaxonera, CRodriguez-Lago, IMesonero, Fde Francisco, RGomez-Gomez, GJChaparro, MTardillo, CARivero, MAlgaba, AArranz, EMCanete, FVicente, RSicilia, BAntolin, BPrieto, VMarquez, LBenitez, JMCamo, PPiqueras, MGargallo, CJHinojosa, EHuguet, JMCalle, JLPVan Domselaar, MRodriguez, CCalvet, XMunoz-Villafranca, CGarcia-Sepulcre, MFMunoz-Garrido, PFernandez-Clotet, AIrwin, LGHernandez, SGuardiola, JSEMPERE, L.Munoza, CGHernandez, VBeltran, BBarrio, JAlba, CMoraleja, ILopez-Sanroman, ARiestra, SMontiel, PMGarre, AArranz, LGarcia, MJArranz, MDMCorsino, PArias, LFernandez-Salazar, LFernandez-Pordomingo, AAndreu, MIglesias, EBer, YMena, RVillarino, MTAMora, MRuiz, LLopez-Serrano, PBlazquez, IVilloria, AFernandez, MBermejo, FBanales, JMDomenech, EGisbert, JPPrimary sclerosing cholangitisinflammatory bowel diseasemalignancyBackground 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.OXFORD UNIV PRESS2019info:eu-repo/semantics/articleinfo:eu-repo/semantics/publishedVersionhttps://fisabio.portalinvestigacion.com/publicaciones/5457Journal of Crohns & ColitisISSN: 18739946ISSNe: 18764479reponame:r-FISABIO. Repositorio Institucional de Producción Científicainstname:Fundación para el Fomento de la Investigación Sanitaria y Biomédica de la Comunitat Valenciana (FISABIO)Inglésinfo:eu-repo/semantics/openAccessoai:fisabio.fundanetsuite.com:p54572026-06-11T12:45:17Z
dc.title.none.fl_str_mv Clinical Characteristics, Associated Malignancies and Management of Primary Sclerosing Cholangitis in Inflammatory Bowel Disease Patients: A Multicentre Retrospective Cohort Study
title Clinical Characteristics, Associated Malignancies and Management of Primary Sclerosing Cholangitis in Inflammatory Bowel Disease Patients: A Multicentre Retrospective Cohort Study
spellingShingle Clinical Characteristics, Associated Malignancies and Management of Primary Sclerosing Cholangitis in Inflammatory Bowel Disease Patients: A Multicentre Retrospective Cohort Study
Guerra, I
Primary sclerosing cholangitis
inflammatory bowel disease
malignancy
title_short Clinical Characteristics, Associated Malignancies and Management of Primary Sclerosing Cholangitis in Inflammatory Bowel Disease Patients: A Multicentre Retrospective Cohort Study
title_full Clinical Characteristics, Associated Malignancies and Management of Primary Sclerosing Cholangitis in Inflammatory Bowel Disease Patients: A Multicentre Retrospective Cohort Study
title_fullStr Clinical Characteristics, Associated Malignancies and Management of Primary Sclerosing Cholangitis in Inflammatory Bowel Disease Patients: A Multicentre Retrospective Cohort Study
title_full_unstemmed Clinical Characteristics, Associated Malignancies and Management of Primary Sclerosing Cholangitis in Inflammatory Bowel Disease Patients: A Multicentre Retrospective Cohort Study
title_sort Clinical Characteristics, Associated Malignancies and Management of Primary Sclerosing Cholangitis in Inflammatory Bowel Disease Patients: A Multicentre Retrospective Cohort Study
dc.creator.none.fl_str_mv Guerra, I
Bujanda, L
Castro, J
Merino, O
Tosca, J
Camps, B
GUTIERREZ, A.
Abalos, JG
de Castro, L
Iborra, M
Carbajo, AY
Taxonera, C
Rodriguez-Lago, I
Mesonero, F
de Francisco, R
Gomez-Gomez, GJ
Chaparro, M
Tardillo, CA
Rivero, M
Algaba, A
Arranz, EM
Canete, F
Vicente, R
Sicilia, B
Antolin, B
Prieto, V
Marquez, L
Benitez, JM
Camo, P
Piqueras, M
Gargallo, CJ
Hinojosa, E
Huguet, JM
Calle, JLP
Van Domselaar, M
Rodriguez, C
Calvet, X
Munoz-Villafranca, C
Garcia-Sepulcre, MF
Munoz-Garrido, P
Fernandez-Clotet, A
Irwin, LG
Hernandez, S
Guardiola, J
SEMPERE, L.
Munoza, CG
Hernandez, V
Beltran, B
Barrio, J
Alba, C
Moraleja, I
Lopez-Sanroman, A
Riestra, S
Montiel, PM
Garre, A
Arranz, L
Garcia, MJ
Arranz, MDM
Corsino, P
Arias, L
Fernandez-Salazar, L
Fernandez-Pordomingo, A
Andreu, M
Iglesias, E
Ber, Y
Mena, R
Villarino, MTA
Mora, M
Ruiz, L
Lopez-Serrano, P
Blazquez, I
Villoria, A
Fernandez, M
Bermejo, F
Banales, JM
Domenech, E
Gisbert, JP
author Guerra, I
author_facet Guerra, I
Bujanda, L
Castro, J
Merino, O
Tosca, J
Camps, B
GUTIERREZ, A.
Abalos, JG
de Castro, L
Iborra, M
Carbajo, AY
Taxonera, C
Rodriguez-Lago, I
Mesonero, F
de Francisco, R
Gomez-Gomez, GJ
Chaparro, M
Tardillo, CA
Rivero, M
Algaba, A
Arranz, EM
Canete, F
Vicente, R
Sicilia, B
Antolin, B
Prieto, V
Marquez, L
Benitez, JM
Camo, P
Piqueras, M
Gargallo, CJ
Hinojosa, E
Huguet, JM
Calle, JLP
Van Domselaar, M
Rodriguez, C
Calvet, X
Munoz-Villafranca, C
Garcia-Sepulcre, MF
Munoz-Garrido, P
Fernandez-Clotet, A
Irwin, LG
Hernandez, S
Guardiola, J
SEMPERE, L.
Munoza, CG
Hernandez, V
Beltran, B
Barrio, J
Alba, C
Moraleja, I
Lopez-Sanroman, A
Riestra, S
Montiel, PM
Garre, A
Arranz, L
Garcia, MJ
Arranz, MDM
Corsino, P
Arias, L
Fernandez-Salazar, L
Fernandez-Pordomingo, A
Andreu, M
Iglesias, E
Ber, Y
Mena, R
Villarino, MTA
Mora, M
Ruiz, L
Lopez-Serrano, P
Blazquez, I
Villoria, A
Fernandez, M
Bermejo, F
Banales, JM
Domenech, E
Gisbert, JP
author_role author
author2 Bujanda, L
Castro, J
Merino, O
Tosca, J
Camps, B
GUTIERREZ, A.
Abalos, JG
de Castro, L
Iborra, M
Carbajo, AY
Taxonera, C
Rodriguez-Lago, I
Mesonero, F
de Francisco, R
Gomez-Gomez, GJ
Chaparro, M
Tardillo, CA
Rivero, M
Algaba, A
Arranz, EM
Canete, F
Vicente, R
Sicilia, B
Antolin, B
Prieto, V
Marquez, L
Benitez, JM
Camo, P
Piqueras, M
Gargallo, CJ
Hinojosa, E
Huguet, JM
Calle, JLP
Van Domselaar, M
Rodriguez, C
Calvet, X
Munoz-Villafranca, C
Garcia-Sepulcre, MF
Munoz-Garrido, P
Fernandez-Clotet, A
Irwin, LG
Hernandez, S
Guardiola, J
SEMPERE, L.
Munoza, CG
Hernandez, V
Beltran, B
Barrio, J
Alba, C
Moraleja, I
Lopez-Sanroman, A
Riestra, S
Montiel, PM
Garre, A
Arranz, L
Garcia, MJ
Arranz, MDM
Corsino, P
Arias, L
Fernandez-Salazar, L
Fernandez-Pordomingo, A
Andreu, M
Iglesias, E
Ber, Y
Mena, R
Villarino, MTA
Mora, M
Ruiz, L
Lopez-Serrano, P
Blazquez, I
Villoria, A
Fernandez, M
Bermejo, F
Banales, JM
Domenech, E
Gisbert, JP
author2_role author
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author
author
author
author
author
author
author
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author
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author
author
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author
author
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author
author
author
author
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author
author
author
author
author
author
author
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dc.subject.none.fl_str_mv Primary sclerosing cholangitis
inflammatory bowel disease
malignancy
topic Primary sclerosing cholangitis
inflammatory bowel disease
malignancy
description 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.
publishDate 2019
dc.date.none.fl_str_mv 2019
dc.type.none.fl_str_mv info:eu-repo/semantics/article
info:eu-repo/semantics/publishedVersion
format article
status_str publishedVersion
dc.identifier.none.fl_str_mv https://fisabio.portalinvestigacion.com/publicaciones/5457
url https://fisabio.portalinvestigacion.com/publicaciones/5457
dc.language.none.fl_str_mv Inglés
language_invalid_str_mv Inglés
dc.rights.none.fl_str_mv info:eu-repo/semantics/openAccess
eu_rights_str_mv openAccess
dc.publisher.none.fl_str_mv OXFORD UNIV PRESS
publisher.none.fl_str_mv OXFORD UNIV PRESS
dc.source.none.fl_str_mv Journal of Crohns & Colitis
ISSN: 18739946
ISSNe: 18764479
reponame:r-FISABIO. Repositorio Institucional de Producción Científica
instname:Fundación para el Fomento de la Investigación Sanitaria y Biomédica de la Comunitat Valenciana (FISABIO)
instname_str Fundación para el Fomento de la Investigación Sanitaria y Biomédica de la Comunitat Valenciana (FISABIO)
reponame_str r-FISABIO. Repositorio Institucional de Producción Científica
collection r-FISABIO. Repositorio Institucional de Producción Científica
repository.name.fl_str_mv
repository.mail.fl_str_mv
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