Objective airway artery dimensions compared to CT scoring methods assessing structural cystic fibrosis lung disease

Abstract 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 A WT A-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 A WT A-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 WT A-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.