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Poor Survival in Rheumatoid Arthritis Associated with Bronchiectasis: A Family-Based Cohort Study
BACKGROUND: Diffuse bronchiectasis (DB) may occur in rheumatoid arthritis (RA). CFTR (cystic fibrosis transmembrane conductance regulator) mutations predispose RA patients to DB, but the prognosis of RA-associated DB (RA-DB) is unclear. METHODS: We report long-term mortality data from a nationwide f...
Autores principales: | , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Public Library of Science
2014
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4195708/ https://www.ncbi.nlm.nih.gov/pubmed/25310716 http://dx.doi.org/10.1371/journal.pone.0110066 |
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author | Puéchal, Xavier Génin, Emmanuelle Bienvenu, Thierry Le Jeunne, Claire Dusser, Daniel J. |
author_facet | Puéchal, Xavier Génin, Emmanuelle Bienvenu, Thierry Le Jeunne, Claire Dusser, Daniel J. |
author_sort | Puéchal, Xavier |
collection | PubMed |
description | BACKGROUND: Diffuse bronchiectasis (DB) may occur in rheumatoid arthritis (RA). CFTR (cystic fibrosis transmembrane conductance regulator) mutations predispose RA patients to DB, but the prognosis of RA-associated DB (RA-DB) is unclear. METHODS: We report long-term mortality data from a nationwide family-based association study of patients with RA only, DB only or RA-DB. We assessed mortality as a function of clinical characteristics and CF/CFTR-RD (CFTR-related disorders) mutations in 137 subjects from 24 kindreds. Potential risk factors were investigated by Cox proportional-hazard analysis with shared Gaussian random effects to account for within-family correlations. RESULTS: During a median follow-up of 11 years after inclusion, 18 patients died, mostly from cardiorespiratory causes. Survival was significantly lower for RA-DB patients than for unaffected relatives and for patients with RA or DB only. RA patients with DB had also a poorer prognosis in terms of survival after RA diagnosis (HR, 8.6; 95% CI, 1.5–48.2; P = 0.014) and from birth (HR, 9.6; 95% CI, 1.1–81.7; P = 0.039). Early onset of DB (HR, 15.4; 95% CI, 2.1–113.2; P = 0.007) and CF/CFTR-RD mutation (HR, 7.2; 95% CI, 1.4–37.1; P = 0.018) were associated with poorer survival in patients with RA-DB. Thus, CF/CFTR-RD mutations in RA patients with early-onset DB defined a subgroup of high-risk patients with higher mortality rates (log-rank test P = 1.28×10(−5)). CONCLUSION: DB is associated with poorer survival in patients with RA. Early-onset DB and CFTR mutations are two markers that identify RA patients at a high risk of death, for whom future therapeutic interventions should be designed and evaluated. |
format | Online Article Text |
id | pubmed-4195708 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2014 |
publisher | Public Library of Science |
record_format | MEDLINE/PubMed |
spelling | pubmed-41957082014-10-15 Poor Survival in Rheumatoid Arthritis Associated with Bronchiectasis: A Family-Based Cohort Study Puéchal, Xavier Génin, Emmanuelle Bienvenu, Thierry Le Jeunne, Claire Dusser, Daniel J. PLoS One Research Article BACKGROUND: Diffuse bronchiectasis (DB) may occur in rheumatoid arthritis (RA). CFTR (cystic fibrosis transmembrane conductance regulator) mutations predispose RA patients to DB, but the prognosis of RA-associated DB (RA-DB) is unclear. METHODS: We report long-term mortality data from a nationwide family-based association study of patients with RA only, DB only or RA-DB. We assessed mortality as a function of clinical characteristics and CF/CFTR-RD (CFTR-related disorders) mutations in 137 subjects from 24 kindreds. Potential risk factors were investigated by Cox proportional-hazard analysis with shared Gaussian random effects to account for within-family correlations. RESULTS: During a median follow-up of 11 years after inclusion, 18 patients died, mostly from cardiorespiratory causes. Survival was significantly lower for RA-DB patients than for unaffected relatives and for patients with RA or DB only. RA patients with DB had also a poorer prognosis in terms of survival after RA diagnosis (HR, 8.6; 95% CI, 1.5–48.2; P = 0.014) and from birth (HR, 9.6; 95% CI, 1.1–81.7; P = 0.039). Early onset of DB (HR, 15.4; 95% CI, 2.1–113.2; P = 0.007) and CF/CFTR-RD mutation (HR, 7.2; 95% CI, 1.4–37.1; P = 0.018) were associated with poorer survival in patients with RA-DB. Thus, CF/CFTR-RD mutations in RA patients with early-onset DB defined a subgroup of high-risk patients with higher mortality rates (log-rank test P = 1.28×10(−5)). CONCLUSION: DB is associated with poorer survival in patients with RA. Early-onset DB and CFTR mutations are two markers that identify RA patients at a high risk of death, for whom future therapeutic interventions should be designed and evaluated. Public Library of Science 2014-10-13 /pmc/articles/PMC4195708/ /pubmed/25310716 http://dx.doi.org/10.1371/journal.pone.0110066 Text en © 2014 Puéchal et al http://creativecommons.org/licenses/by/4.0/ This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are properly credited. |
spellingShingle | Research Article Puéchal, Xavier Génin, Emmanuelle Bienvenu, Thierry Le Jeunne, Claire Dusser, Daniel J. Poor Survival in Rheumatoid Arthritis Associated with Bronchiectasis: A Family-Based Cohort Study |
title | Poor Survival in Rheumatoid Arthritis Associated with Bronchiectasis: A Family-Based Cohort Study |
title_full | Poor Survival in Rheumatoid Arthritis Associated with Bronchiectasis: A Family-Based Cohort Study |
title_fullStr | Poor Survival in Rheumatoid Arthritis Associated with Bronchiectasis: A Family-Based Cohort Study |
title_full_unstemmed | Poor Survival in Rheumatoid Arthritis Associated with Bronchiectasis: A Family-Based Cohort Study |
title_short | Poor Survival in Rheumatoid Arthritis Associated with Bronchiectasis: A Family-Based Cohort Study |
title_sort | poor survival in rheumatoid arthritis associated with bronchiectasis: a family-based cohort study |
topic | Research Article |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4195708/ https://www.ncbi.nlm.nih.gov/pubmed/25310716 http://dx.doi.org/10.1371/journal.pone.0110066 |
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