Impact of bronchiectasis and trapped air on quality of life and exacerbations in cystic fibrosis

Leonie A. Tepper, Elisabeth M.W.J. Utens, Daan Caudri, Aukje C. Bos, Karla Gonzalez-Graniel, Hugo J. Duivenvoorden, Els C.W. Van Der Wiel, Alexandra L. Quittner, Harm A.W.M. Tiddens

Research output: Contribution to journalArticle

34 Scopus citations

Abstract

Cystic fibrosis (CF) is primarily characterised by bronchiectasis and trapped air on chest computed tomography (CT). The revised Cystic Fibrosis Questionnaire respiratory symptoms scale (CFQ-R RSS) measures health-related quality of life. To validate bronchiectasis, trapped air and CFQ-R RSS as outcome measures, we investigated correlations and predictive values for pulmonary exacerbations. CF patients (aged 6-20 years) underwent CT, CFQ-R RSS and 1-year follow-up. Bronchiectasis and trapped air were scored using the CF-CT scoring system. Correlation coefficients and backward multivariate modelling were used to identify predictors of pulmonary exacerbations. 40 children and 32 adolescents were included. CF-CT bronchiectasis (r=-0.38, p<0.001) and CF-CT trapped air (r=-0.35, p=0.003) correlated with CFQ-R RSS. Pulmonary exacerbations were associated with: bronchiectasis (rate ratio 1.10, 95% CI 1.02-1.19; p=0.009), trapped air (rate ratio 1.02, 95% CI 1.00- 1.05; p=0.034) and CFQ-R RSS (rate ratio 0.95, 95% CI 0.91-0.98; p=0.002). The CFQ-R RSS was an independent predictor of pulmonary exacerbations (rate ratio 0.96, 95% CI 0.94-0.97; p<0.001). Bronchiectasis, trapped air and CFQ-R RSS were associated with pulmonary exacerbations. The CFQ-R RSS was an independent predictor. This study further validated bronchiectasis, trapped air and CFQ-R RSS as outcome measures in CF.

Original languageEnglish (US)
Pages (from-to)371-379
Number of pages9
JournalEuropean Respiratory Journal
Volume42
Issue number2
DOIs
StatePublished - Aug 1 2013

    Fingerprint

ASJC Scopus subject areas

  • Pulmonary and Respiratory Medicine

Cite this

Tepper, L. A., Utens, E. M. W. J., Caudri, D., Bos, A. C., Gonzalez-Graniel, K., Duivenvoorden, H. J., Van Der Wiel, E. C. W., Quittner, A. L., & Tiddens, H. A. W. M. (2013). Impact of bronchiectasis and trapped air on quality of life and exacerbations in cystic fibrosis. European Respiratory Journal, 42(2), 371-379. https://doi.org/10.1183/09031936.00137612