Abstract
ABSTRACT Background: Computed tomography (CT) of the lungs is the gold standard for assessing the extent of structural changes in the lungs. Spirometry-controlled chest CT (SCCCT) has improved the usefulness of CT by standardising inspiratory and expiratory lung volumes during imaging. This was a single-centre cross-sectional study in children with cystic fibrosis (CF). Using SCCCT we wished to investigate the association between the quantity and extent of structural lung changes and pulmonary function outcomes, and prevalence of known CF lung pathogens. Methods: CT images were analysed by CF-CT scoring (expressed as % of maximum score) to quantify different aspects of structural lung changes including bronchiectasis, airway wall thickening, mucus plugging, opacities, cysts, bullae and gas trapping. Clinical markers consisted of outcomes from pulmonary function tests, microbiological cultures from sputum and serological samples reflecting anti-bacterial and anti-fungal antibodies. Results: Sixty-four children with CF, median age (range) of 12.7 (6.4–18.1) years, participated in the study. The median (range) CF-CT total score in all children was 9.3% (0.4–46.8) with gas trapping of 40.7% (3.7–100) as the most abundant finding. Significantly higher median CF-CT total scores (21.9%) were found in patients with chronic infections (N = 12) including Gram-negative infection and allergic bronchopulmonary aspergillosis (ABPA) exhibiting CF-CT total scores of 14.2% (ns) and 24.0% (p < 0.01), respectively, compared to 8.0% in patients with no chronic lung infection. Lung clearance index (LCI) derived from multiple breath washout exhibited closest association with total CF-CT scores, compared to other pulmonary function outcomes. Conclusions: The most prominent structural lung change was gas trapping, while CF-CT total scores were generally low, both showing close association with LCI. Chronic lung infections, specifically in the form of ABPA, were associated with increased scores in lung changes. Further investigation of impact of infections with different microorganisms on extent and progression of structural CF lung disease is needed.
Highlights
Structural lung changes such as bronchiectasis, airway wall thickening, mucus plugging, opacities, cysts, bullae and gas trapping are very common in patients with cystic fibrosis (CF) and caused by a vicious cycle of mucus stagnation, inflammation, and infection.[1,2] Progression of structural lung changes is a sign of worsening lung disease that will eventually lead to increased morbidity and mortality.[3]
Computed tomography (CT) of the lungs is the gold standard for assessing the extent of structural changes in the lungs,[4,5] and manoeuvres for controlling breath holds during imaging has further improved the usefulness of CT by standardising lung volumes
The purpose of this study was to investigate the association between the quantity and extent of structural lung changes using refined spirometry controlled chest computed tomography (SCCCT) with computeranimated feedback and clinical markers of CF lung disease such as pulmonary function outcomes, and prevalence of known CF lung pathogens in a cohort of clinically stable CF children
Summary
Structural lung changes such as bronchiectasis, airway wall thickening, mucus plugging, opacities, cysts, bullae and gas trapping are very common in patients with cystic fibrosis (CF) and caused by a vicious cycle of mucus stagnation, inflammation, and infection.[1,2] Progression of structural lung changes is a sign of worsening lung disease that will eventually lead to increased morbidity and mortality.[3]. Computed tomography (CT) of the lungs is the gold standard for assessing the extent of structural changes in the lungs,[4,5] and manoeuvres for controlling breath holds during imaging has further improved the usefulness of CT by standardising lung volumes. This standardisation improves the cross-sectional and longitudinal comparability between groups and within subjects with repeated examinations,[6,7,8] and increases the recognition of gas trapping.[9]. Further investigation of impact of infections with different microorganisms on extent and progression of structural CF lung disease is needed
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