Abstract
BackgroundDiffuse 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.MethodsWe 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.ResultsDuring 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).ConclusionDB 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.
Highlights
Rheumatoid arthritis (RA) is a systemic autoimmune disease, of unknown origin, that affects 0.5–1.0% of the adult population worldwide [1]
In prospective blind studies based on highresolution computed tomography (HRCT) in patients with rheumatoid arthritis (RA), the prevalence of diffuse bronchiectasis has been reported to range from 5.6% to 30% [4,5,6,7,8,9]
We previously reported a higher prevalence of cystic fibrosis (CF)/CFTR-related diseases (CFTR-RD) mutations in patients suffering from RA-associated Diffuse bronchiectasis (DB) (RA-DB) than in the general population [16]
Summary
Rheumatoid arthritis (RA) is a systemic autoimmune disease, of unknown origin, that affects 0.5–1.0% of the adult population worldwide [1]. It primarily involves the joints, but extra-articular manifestations, such as lung involvement, have been observed and may increase the burden of this frequently disabling disease. In less than 10% of patients, respiratory symptoms of DB occur concomitantly with the onset of arthritis (late-onset and adulthood DB) [17]. The prognosis of these two subtypes of DB in RA patients remains unclear. CFTR (cystic fibrosis transmembrane conductance regulator) mutations predispose RA patients to DB, but the prognosis of RA-associated DB (RA-DB) is unclear
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