RT Journal Article
SR Electronic
T1 Lung clearance index predicts pulmonary exacerbations in cystic fibrosis
JF European Respiratory Journal
JO Eur Respir J
FD European Respiratory Society
SP P1219
VO 44
IS Suppl 58
A1 Joerg Grosse-Onnebrink
A1 Florian Stehling
A1 Uwe Mellies
YR 2014
UL http://erj.ersjournals.com/content/44/Suppl_58/P1219.abstract
AB Background:The lung clearance index (LCI) is increasingly used as an endpoint in clinical trials, but data showing its associations with clinical endpoints and its prognostic value are spares.Objectives:We aimed to show whether the LCI is associated with clinical parameters supposed to reflect lung disease in cystic fibrosis and whether the LCI predicts subsequent pulmonary exacerbations (PE).Methods:Baseline LCI, spirometry parameters and clinical data were obtained and PE per year were prospectively recorded in 59 patients with proven cystic fibrosis. Linear regression models were used to explore associations between baseline data and LCI and between LCI and PE.Results:59 Patients with a mean age of 17.8 years (range 6.3; 44.6) with a mean baseline FEV1 of -3.1 z-score (-13.2; 2.7) and a mean LCI of 9.9 (6.2; 18.6) were evaluated for a mean of 3.1 years (1.5; 3.5) years. 165 PE were recorded, the mean annual PE rate was 2.8 (0; 14). PO2 (β =-0.124; 95%CI -0.188; -0.060), FEV1 z-score (β= -0.288 95%CI -0.534; -0.041) and status of infection with Pseudomonas aeruginosa (β= -1,759 95%CI -3.029; -0.489) were independent predictors of LCI (r=0.779; r2=0.606). LCI was the only predictor of subsequent PE (β=0.306 95%CI 0.196; 0.415, r=0.629; r2=0.40).Conclusion:We show that the LCI is associated with surrogate parameters of lung disease. By predicting PE the LCI is also associated with markers of clinical deterioration in cystic fibrosis patients. Our data support the use of LCI as a valuable endpoint in clinical trials.