Progression of structural lung disease and lung function in adolescents with cystic fibrosis.
Authors
Affiliations (10)
Affiliations (10)
- Department of Paediatrics, Division of Respiratory Medicine and Allergology, Sophia Children's Hospital, Erasmus MC, Rotterdam, The Netherlands; Department of Radiology and Nuclear Medicine, Erasmus MC, Rotterdam, The Netherlands.
- Department of Biostatistics, Erasmus MC, Rotterdam, Netherlands; Department of Epidemiology, Erasmus MC, Rotterdam, Netherlands.
- Starship Children's Health and Department of Paediatrics, University of Auckland, Auckland, New Zealand.
- Department of Respiratory and Sleep Medicine, Queensland Children's Hospital, Brisbane, Australia; Child Health Research Centre, The University of Queensland, Brisbane, Australia.
- Department of Respiratory and Sleep Medicine, The Children's Hospital at Westmead, Sydney, Australia.
- School of Medicine and Dentistry, Griffith University, Gold Coast, Australia.
- Department of Respiratory Medicine, Royal Children's Hospital, Melbourne, Australia.
- Department of Paediatrics, University of Melbourne, Melbourne, Australia.
- Department of Paediatrics, University of Melbourne, Melbourne, Australia; Clinical Epidemiology and Biostatistics Unit, Murdoch Children's Research Institute, Melbourne, Australia.
- Department of Paediatrics, Division of Respiratory Medicine and Allergology, Sophia Children's Hospital, Erasmus MC, Rotterdam, The Netherlands; Department of Radiology and Nuclear Medicine, Erasmus MC, Rotterdam, The Netherlands; Thirona, Nijmegen, The Netherlands. Electronic address: [email protected].
Abstract
Cystic fibrosis (CF) lung disease begins early in life and progresses throughout childhood into adolescence. Children completing the Australasian CF Bronchoalveolar Lavage (ACFBAL) trial were followed longitudinally (CF-FAB study) to determine progression of CF lung disease during adolescence using visual and automatic methods and to correlate CT-derived metrics with spirometry outcomes. CTs from start and end visits of CF-FAB (mean 24 [SD 12] months apart) were analysed using visual PRAGMA-CF scoring and automatic bronchus-artery (BA) analysis. PRAGMA-CF assessed %Disease by summing %Bronchiectasis, %Mucus plugging, % Airway wall thickening on inspiratory scans and %Trapped air on expiratory scans. The BA-analysis segments the bronchial tree, identifies segmental bronchi (G<sub>0</sub>) and distal generations (G<sub>1</sub>, G<sub>2</sub>, G<sub>3</sub>…), measures diameters of bronchial outer wall (B<sub>out</sub>), inner wall (B<sub>in</sub>), wall thickness (B<sub>wt</sub>), and artery (A), and computes BA-ratios (B<sub>out</sub>/A, B<sub>in</sub>/A, B<sub>wt</sub>/A, B<sub>wa</sub>/B<sub>oa</sub>[=bronchial wall area/bronchial outer area]) to evaluate bronchial dilatation and wall thickening. 120 children (median age 13 years, IQR 11.4-14) contributed 115 start and 105 end scans. Eleven children were treated with CFTR modulators prior to the start of the study and four received the treatment during the study. Progression was found in PRAGMA-CF %Bronchiectasis (p=0.02) and %Mucus plugging (p=0.02), and B<sub>out</sub>/A (p=0.01), B<sub>wt</sub>/A (p<0.001), and B<sub>wa</sub>/B<sub>oa</sub> (p<0.001). Spirometry outcomes showed no significant decline. BA-metrics correlated more strongly with spirometry outcomes than PRAGMA-CF scores. Heterogeneous progression of structural lung disease in children with CF during adolescence was detected using visual PRAGMA-CF scores and automatic BA-analysis. Spirometry outcomes showed no significant decline.