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

Eur Respir J. 2013 Aug;42(2):371-9. doi: 10.1183/09031936.00137612. Epub 2013 Jan 11.

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.

Publication types

  • Validation Study

MeSH terms

  • Adolescent
  • Air
  • Bronchiectasis / complications*
  • Bronchiectasis / diagnostic imaging
  • Bronchiectasis / psychology*
  • Child
  • Cystic Fibrosis / complications*
  • Cystic Fibrosis / diagnostic imaging
  • Cystic Fibrosis / psychology*
  • Female
  • Humans
  • Lung / diagnostic imaging
  • Male
  • Multivariate Analysis
  • Mutation
  • Observer Variation
  • Quality of Life*
  • Retrospective Studies
  • Surveys and Questionnaires
  • Tomography, X-Ray Computed / methods
  • Treatment Outcome
  • Young Adult