Clinical Characteristics, Associated Malignancies and Management of Primary Sclerosing Cholangitis in Inflammatory Bowel Disease Patients: A Multicentre Retrospective Cohort Study
Por:
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 and Gisbert, JP
Publicada:
1 dic 2019
Resumen:
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.
Filiaciones:
Guerra, I:
Hosp Univ Fuenlabrada, Madrid, Spain
Hosp Univ La Paz, Inst Invest, Madrid, Spain
Bujanda, L:
Univ Basque Country, UPV EHU, Inst Biodonostia, Hosp Donostia, Donostia San Sebastian, Spain
Ctr Invest Biomed Red Emfermedades Hepat & Digest, Madrid, Spain
Castro, J:
Hosp Clin Barcelona, Barcelona, Spain
Merino, O:
Hosp Univ Cruces, Baracaldo, Vizcaya, Spain
Tosca, J:
Univ Valencia, Dept Med, Hosp Univ Clin Valencia, Valencia, Spain
Camps, B:
Hosp Univ Bellvitge, Inst Invest Biomed Bellvitge IDIBELL, Lhospitalet De Llobregat, Spain
Gutierrez, A:
Ctr Invest Biomed Red Emfermedades Hepat & Digest, Madrid, Spain
Hosp Gen Univ Alicante, Alicante, Spain
Abalos, JG:
Hosp Santa Creu & Sant Pau, Barcelona, Spain
de Castro, L:
Complexo Hosp Univ Vigo, Inst Invest Biomed, Estruct Org Xest Integrada Vigo, Vigo, Spain
Iborra, M:
Ctr Invest Biomed Red Emfermedades Hepat & Digest, Madrid, Spain
Hosp Univ & Politecn La Fe, Valencia, Spain
Carbajo, AY:
Hosp Univ Rio Hortega, Valladolid, Spain
Taxonera, C:
Hosp Clin San Carlos, Madrid, Spain
Hosp Clin San Carlos IdISSC, Inst Invest, Madrid, Spain
Rodriguez-Lago, I:
Hosp Galdakao, Galdakao, Vizcaya, Spain
Mesonero, F:
Hosp Univ Ramon y Cajal, Madrid, Spain
de Francisco, R:
Hosp Univ Cent Asturias, Inst Invest Sanitaria Principado Asturias ISPA, Oviedo, Spain
Gomez-Gomez, GJ:
Hosp 12 Octubre, Madrid, Spain
Chaparro, M:
Ctr Invest Biomed Red Emfermedades Hepat & Digest, Madrid, Spain
Univ Autonoma Madrid, Inst Invest Sanitaria Princesa IIS IP, Hosp Univ La Princesa, Madrid, Spain
Tardillo, CA:
Hosp Univ Nuestra Senora Candelaria, Santa Cruz de Tenerife, Spain
Rivero, M:
Hosp Univ Marques Valdecilla, Santander, Spain
IDIVAL, Santander, Spain
Algaba, A:
Hosp Univ Fuenlabrada, Madrid, Spain
Hosp Univ La Paz, Inst Invest, Madrid, Spain
Arranz, EM:
Hosp Univ La Paz, Madrid, Spain
Hosp Univ La Paz, Inst Invest, IdiPaz, Madrid, Spain
:
Ctr Invest Biomed Red Emfermedades Hepat & Digest, Madrid, Spain
Hosp Badalona Germans Trias & Pujol, Badalona, Spain
Vicente, R:
Hosp Univ Miguel Servet, Zaragoza, Spain
Sicilia, B:
Hosp Univ Burgos, Burgos, Spain
Antolin, B:
Hosp Clin Univ Valladolid, Valladolid, Spain
Prieto, V:
Hosp Univ Salamanca, Salamanca, Spain
Marquez, L:
Parc Salut Mar, Barcelona, Spain
Benitez, JM:
Hosp Univ Reina Sofia, Cordoba, Spain
Inst Maimonides Invest Biomed Cordoba IMIBIC, Cordoba, Spain
Camo, P:
Hosp Gen San Jorge, Huesca, Spain
Piqueras, M:
Consorci Sanitari Terrassa, Terrassa, Spain
Gargallo, CJ:
Hosp Clin Univ Lozano Blesa, Zaragoza, Spain
Aragon Hlth Res Inst IIS Aragon, Zaragoza, Spain
Hinojosa, E:
Hosp Manises, Valencia, Spain
Huguet, JM:
Hosp Gen Univ Valencia, Valencia, Spain
Calle, JLP:
Hosp Univ Fdn Alcorcon, Madrid, Spain
Van Domselaar, M:
Hosp Univ Torrejon, Madrid, Spain
Univ Francisco Vitoria, Madrid, Spain
Rodriguez, C:
Complejo Hosp Navarra, Pamplona, Spain
Calvet, X:
Ctr Invest Biomed Red Emfermedades Hepat & Digest, Madrid, Spain
Univ Autonoma Barcelona, Corp Sanitaria Univ Parc Tauli, Sabadell, Spain
Munoz-Villafranca, C:
Hosp Univ Basurto, Bilbao, Spain
Garcia-Sepulcre, MF:
Hosp Gen Univ Elche, Alicante, Spain
Munoz-Garrido, P:
Univ Basque Country, UPV EHU, Inst Biodonostia, Hosp Donostia, Donostia San Sebastian, Spain
Ctr Invest Biomed Red Emfermedades Hepat & Digest, Madrid, Spain
Fernandez-Clotet, A:
Hosp Clin Barcelona, Barcelona, Spain
Irwin, LG:
Hosp Univ Cruces, Baracaldo, Vizcaya, Spain
Hernandez, S:
Univ Valencia, Dept Med, Hosp Univ Clin Valencia, Valencia, Spain
Guardiola, J:
Hosp Univ Bellvitge, Inst Invest Biomed Bellvitge IDIBELL, Lhospitalet De Llobregat, Spain
Univ Barcelona, Barcelona, Spain
Sempere, L:
Hosp Gen Univ Alicante, Alicante, Spain
Munoza, CG:
Hosp Santa Creu & Sant Pau, Barcelona, Spain
Hernandez, V:
Complexo Hosp Univ Vigo, Inst Invest Biomed, Estruct Org Xest Integrada Vigo, Vigo, Spain
Beltran, B:
Ctr Invest Biomed Red Emfermedades Hepat & Digest, Madrid, Spain
Hosp Univ & Politecn La Fe, Valencia, Spain
Barrio, J:
Hosp Univ Rio Hortega, Valladolid, Spain
Alba, C:
Hosp Clin San Carlos, Madrid, Spain
Hosp Clin San Carlos IdISSC, Inst Invest, Madrid, Spain
Moraleja, I:
Hosp Galdakao, Galdakao, Vizcaya, Spain
Lopez-Sanroman, A:
Hosp Univ Ramon y Cajal, Madrid, Spain
Riestra, S:
Hosp Univ Cent Asturias, Inst Invest Sanitaria Principado Asturias ISPA, Oviedo, Spain
Montiel, PM:
Hosp 12 Octubre, Madrid, Spain
Garre, A:
Ctr Invest Biomed Red Emfermedades Hepat & Digest, Madrid, Spain
Univ Autonoma Madrid, Inst Invest Sanitaria Princesa IIS IP, Hosp Univ La Princesa, Madrid, Spain
Arranz, L:
Hosp Univ Nuestra Senora Candelaria, Santa Cruz de Tenerife, Spain
Garcia, MJ:
Hosp Univ Marques Valdecilla, Santander, Spain
IDIVAL, Santander, Spain
Arranz, MDM:
Hosp Univ La Paz, Madrid, Spain
Hosp Univ La Paz, Inst Invest, IdiPaz, Madrid, Spain
Corsino, P:
Hosp Univ Miguel Servet, Zaragoza, Spain
Arias, L:
Hosp Univ Burgos, Burgos, Spain
Fernandez-Salazar, L:
Hosp Clin Univ Valladolid, Valladolid, Spain
Fernandez-Pordomingo, A:
Hosp Univ Salamanca, Salamanca, Spain
Andreu, M:
Parc Salut Mar, Barcelona, Spain
Iglesias, E:
Hosp Univ Reina Sofia, Cordoba, Spain
Inst Maimonides Invest Biomed Cordoba IMIBIC, Cordoba, Spain
Ber, Y:
Hosp Gen San Jorge, Huesca, Spain
Mena, R:
Consorci Sanitari Terrassa, Terrassa, Spain
Villarino, MTA:
Hosp Clin Univ Lozano Blesa, Zaragoza, Spain
Aragon Hlth Res Inst IIS Aragon, Zaragoza, Spain
Mora, M:
Hosp Manises, Valencia, Spain
Ruiz, L:
Hosp Gen Univ Valencia, Valencia, Spain
Lopez-Serrano, P:
Hosp Univ Fdn Alcorcon, Madrid, Spain
Blazquez, I:
Hosp Univ Torrejon, Madrid, Spain
Univ Francisco Vitoria, Madrid, Spain
Villoria, A:
Ctr Invest Biomed Red Emfermedades Hepat & Digest, Madrid, Spain
Complejo Hosp Navarra, Pamplona, Spain
Fernandez, M:
Univ Autonoma Barcelona, Corp Sanitaria Univ Parc Tauli, Sabadell, Spain
Bermejo, F:
Hosp Univ Fuenlabrada, Madrid, Spain
Hosp Univ La Paz, Inst Invest, Madrid, Spain
Banales, JM:
Univ Basque Country, UPV EHU, Inst Biodonostia, Hosp Donostia, Donostia San Sebastian, Spain
Ctr Invest Biomed Red Emfermedades Hepat & Digest, Madrid, Spain
Ikerbasque, Basque Fdn Sci, Bilbao, Spain
:
Ctr Invest Biomed Red Emfermedades Hepat & Digest, Madrid, Spain
Hosp Badalona Germans Trias & Pujol, Badalona, Spain
Gisbert, JP:
Ctr Invest Biomed Red Emfermedades Hepat & Digest, Madrid, Spain
Univ Autonoma Madrid, Inst Invest Sanitaria Princesa IIS IP, Hosp Univ La Princesa, Madrid, Spain
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