Background: CF-CT and PRAGMA-CF are commonly used scoring methods to quantify the severity of bronchiectasis (BE) and airway wall thickening (AWT) on chest CTs of children with cystic fibrosis (CF). We aimed to validate CF-CT and PRAGMA-CF sub-scores for BE and AWT against quantitative airway-artery (AA) dimensions. Methods: This is a retrospective study with 23 spirometer guided inspiratory chest CTs (11 CF, 12 controls; age range 6 to 16years old) included. AA-, and AWTA-ratios of all visible AA pairs were computed by dividing diameters of the outer airway and wall (outer-inner airway) by the accompanying artery diameter, respectively. BE, AWT and total airway disease (TAD) were scored using CF-CT (% max score) and PRAGMA-CF (% extent). Correlations were computed using Spearman rank. Akaike information criterion (AIC) from the mixed-effects models were used to investigate whether CF-CT or PRAGMA-CF was a better predictor for AA-, and AWTA-ratios (lower AIC equals a better fitted model). Results: 4861 AA pairs were measured in total. Correlations between CF-CT and PRAGMA-CF: BE (r=0.93, P<0.001); AWT (r=0.62, P<0.001); TAD (r=0.88, P<0.001). PRAGMA-CF TAD sub-score had lowest AIC in the mixed-model predicting AA-ratio. CF-CT AWT and PRAGMA-CF TAD sub-score had equal low AIC in the mixed-model predicting AWTA-ratio. Conclusion: PRAGMA-CF TAD sub-score was more precise predicting BE. CF-CT AWT and PRAGMA-CF TAD sub-scores predicted AWT equally well. CF-CT and PRAGMA-CF were both sensitive methods to score BE and AWT in children with CF lung disease, with PRAGMA-CT TAD sub-score being most accurate in predicting AA dimensions.

, , , ,,
Journal of Cystic Fibrosis
Department of Pediatrics

Kuo-Kim, W., Andrinopoulou, E.-R., Perez-Rovira, A., Ozturk, H. (Hadiye), de Bruijne, M., & Tiddens, H.H.A.W.M. (Harm H.A.W.M.). (2017). Objective airway artery dimensions compared to CT scoring methods assessing structural cystic fibrosis lung disease. Journal of Cystic Fibrosis, 16(1), 116–123. doi:10.1016/j.jcf.2016.05.015