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 journalArticlepeer-review

38 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

ASJC Scopus subject areas

  • Pulmonary and Respiratory Medicine

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