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

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 (r5 -0.38, p,0.001) and CF-CT trapped air (r5 -0.35, p50.003) correlated with CFQ-R RSS. Pulmonary exacerbations were associated with: bronchiectasis (rate ratio 1.10, 95% CI 1.02-1.19; p50.009), trapped air (rate ratio 1.02, 95% CI 1.00-1.05; p50.034) and CFQ-R RSS (rate ratio 0.95, 95% CI 0.91-0.98; p50.002). 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. @ERSpublications A further validation of bronchiectasis and trapped air on CT and CFQ-R respiratory symptoms as outcome measures in CF

Blood basophil activation is a reliable biomarker of allergic bronchopulmonary aspergillosis in cystic fibrosis

“…Another limitation of the CD203c BAT is that it may be difficult to discriminate patients sensitised to A. fumigatus from patients with CF-ABPA, but we believe additional analysis of A. fumigatus-specific IgE levels may be able to resolve this issue in most cases [22]. Finally, it has been shown that HRCT can detect pulmonary exacerbations in CF patients [37]. As we did not design our study to compare BAT CD203c levels in patients with CF and ABPA to chest HRCT bronchiectasis scores, future long-term studies will be necessary to assess the potential correlation between the persistence of basophil CD203c activation in patients with CF-ABPA and progression of bronchiectasis on chest HRCT.…”

Assessment of CF lung disease using motion corrected PROPELLER MRI: a comparison with CT

“…Using chest CT as the reference test, a cut-off value of 26 % or higher, representing severe bronchiectasis, was defined as a true-positive finding for the MRI-bronchiectasis subscore; 26 % represents the percentage of the maximum possible score for bronchiectasis, which is 12 per each lobe and 72 in total according to CF-CT. This cut-off of 26 % was based on the median value of bronchiectasis severity observed in two cohort studies, with either mild or severe CF lung disease [3,22], using receiveroperating characteristic analysis to determine different cut-off values. Sensitivity, specificity, PPV and NPV were calculated for each observer (obs 1 and obs 2 ), in addition to the mean of the bronchiectasis scores of both observers (mean obs 1+2 ).…”

“…Life expectancy is reduced because of progressive lung disease, characterised by several structural lung changes, most importantly bronchiectasis and trapped air [2,3]. To guide therapy to prevent or reduce bronchiectasis and trapped air, it is crucial to monitor CF lung disease at regular intervals.…”

Air Trapping and Airflow Obstruction in Newborn Cystic Fibrosis Piglets

“…Moreover, the existence of air trapping in infants with CF is predictive of future pulmonary exacerbations (41) and is a risk factor for bronchiectasis (28). In newborn piglets with CF, air trapping cannot be attributed to mucus plugging, infection, inflammation, or their secondary consequences, because these changes are not present (10,12,15).…”