Phenotypic concordance in familial inflammatory bowel disease (IBD). Results of a nationwide IBD Spanish database
Por:
Cabre, E, Manosa, M, Garcia-Sanchez, V, Gutierrez, A, Ricart, E, Esteve, M, Guardiola, J, Aguas, M, Merino, O, Ponferrada, A, Gisbert, JP, Garcia-Planella, E, Cena, G, Cabriada, JL, Montoro, M, Domenech, E
Publicada:
1 jul 2014
Resumen:
Background & aims: Disease outcome has been found to be poorer in familial inflammatory bowel disease (IBD) than in sporadic forms, but assessment of phenotypic concordance in familial IBD provided controversial results. We assessed the concordance for disease type and phenotypic features in IBD families.
Methods: Patients with familial IBD were identified from the IBD Spanish database ENEIDA. Families in whom at least two members were in the database were selected for concordance analysis (K index). Concordance for type of IBD [Crohn's disease (CD) vs. ulcerative colitis (UC)], as well as for disease extent, localization and behaviour, perianal disease, extraintestinal manifestations, and indicators of severe disease (i.e., need for immunosuppressors, biological agents, and surgery) for those pairs concordant for IBD type, were analyzed.
Results: 798 out of 11,905 IBD patients (7%) in ENEIDA had familial history of IBD. Complete data of 107 families (231 patients and 144 consanguineous pairs) were available for concordance analyses. The youngest members of the pairs were diagnosed with IBD at a significantly younger age (p < 0.001) than the oldest ones. Seventy-six percent of pairs matched up for the IBD type (kappa = 0.58; 95%CI: 0.42-0.73, moderate concordance). There was no relevant concordance for any of the phenotypic items assessed in both diseases.
Conclusions: Familial IBD is associated with diagnostic anticipation in younger individuals. Familial history does not allow predicting any phenotypic feature other than IBD type. (C) 2013 European Crohn's and Colitis Organisation. Published by Elsevier B.V. All rights reserved.
Filiaciones:
Cabre, E:
Hosp Badalona Germans Trias & Pujol, Badalona 08916, Catalonia, Spain
Ctr Invest Biomed Red Enfermedades Hepat & Digest, Madrid, Spain
Manosa, M:
Hosp Badalona Germans Trias & Pujol, Badalona 08916, Catalonia, Spain
Ctr Invest Biomed Red Enfermedades Hepat & Digest, Madrid, Spain
Garcia-Sanchez, V:
Hosp Univ Reina Sofia, Cordoba, Spain
Gutierrez, A:
Hosp Gen Univ, Alicante, Spain
Ricart, E:
Hosp Clin Barcelona, Barcelona, Spain
Ctr Invest Biomed Red Enfermedades Hepat & Digest, Madrid, Spain
Esteve, M:
Hosp Univ Mutua Terrassa, Terrassa, Spain
Ctr Invest Biomed Red Enfermedades Hepat & Digest, Madrid, Spain
Guardiola, J:
Hosp Llobregat, Hosp Univ Bellvitge, Barcelona, Spain
Aguas, M:
Hosp Univ La Fe, Valencia, Spain
Ctr Invest Biomed Red Enfermedades Hepat & Digest, Madrid, Spain
Merino, O:
Hosp Cruces, Baracaldo, Spain
Ponferrada, A:
Hosp Univ Infanta Leonor, Madrid, Spain
Gisbert, JP:
Hosp Univ La Princesa, Madrid, Spain
Ctr Invest Biomed Red Enfermedades Hepat & Digest, Madrid, Spain
Garcia-Planella, E:
Hosp Santa Creu i St Pau, Barcelona, Spain
Cena, G:
Hosp Royo Villanova, Zaragoza, Spain
Cabriada, JL:
Hosp Galdakao Usansolo, Galdakao, Spain
Montoro, M:
Hosp Gen San Jorge, Huesca, Spain
Domenech, E:
Hosp Badalona Germans Trias & Pujol, Badalona 08916, Catalonia, Spain
Ctr Invest Biomed Red Enfermedades Hepat & Digest, Madrid, Spain
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