Poor survival in rheumatoid arthritis associated with bronchiectasis: a family-based cohort study.

<h4>Background</h4>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.<h4>Methods</h4>We report long-te...

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Autores principales:	Xavier Puéchal, Emmanuelle Génin, Thierry Bienvenu, Claire Le Jeunne, Daniel J Dusser
Formato:	article
Lenguaje:	EN
Publicado:	Public Library of Science (PLoS) 2014
Materias:	Medicine R Science Q
Acceso en línea:	https://doaj.org/article/212b47eb74ea49018c162c0c2a1e04cb
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spelling	oai:doaj.org-article:212b47eb74ea49018c162c0c2a1e04cb2021-11-25T05:56:49ZPoor survival in rheumatoid arthritis associated with bronchiectasis: a family-based cohort study.1932-620310.1371/journal.pone.0110066https://doaj.org/article/212b47eb74ea49018c162c0c2a1e04cb2014-01-01T00:00:00Zhttps://doi.org/10.1371/journal.pone.0110066https://doaj.org/toc/1932-6203<h4>Background</h4>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.<h4>Methods</h4>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.<h4>Results</h4>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)).<h4>Conclusion</h4>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.Xavier PuéchalEmmanuelle GéninThierry BienvenuClaire Le JeunneDaniel J DusserPublic Library of Science (PLoS)articleMedicineRScienceQENPLoS ONE, Vol 9, Iss 10, p e110066 (2014)
institution	DOAJ
collection	DOAJ
language	EN
topic	Medicine R Science Q
spellingShingle	Medicine R Science Q Xavier Puéchal Emmanuelle Génin Thierry Bienvenu Claire Le Jeunne Daniel J Dusser Poor survival in rheumatoid arthritis associated with bronchiectasis: a family-based cohort study.
description	<h4>Background</h4>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.<h4>Methods</h4>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.<h4>Results</h4>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)).<h4>Conclusion</h4>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	article
author	Xavier Puéchal Emmanuelle Génin Thierry Bienvenu Claire Le Jeunne Daniel J Dusser
author_facet	Xavier Puéchal Emmanuelle Génin Thierry Bienvenu Claire Le Jeunne Daniel J Dusser
author_sort	Xavier Puéchal
title	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_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_sort	poor survival in rheumatoid arthritis associated with bronchiectasis: a family-based cohort study.
publisher	Public Library of Science (PLoS)
publishDate	2014
url	https://doaj.org/article/212b47eb74ea49018c162c0c2a1e04cb
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Poor survival in rheumatoid arthritis associated with bronchiectasis: a family-based cohort study.

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