Monitoring of Cystic Fibrosis Lung Disease Using Computed Tomography

For clinical management of cystic fibrosis (CF) lung disease to be effective, onset and worsening of lung abnormalities should be closely monitored. Pulmonary function tests (PFTs) are currently the gold standard to monitor CF lung disease. Lung structure can be more sensitively monitored using computed tomography (CT) rather than chest radiography. Firstly, we compared in two pediatric cohorts the sensitivities of CT and PFTs to detect onset and worsening of CF lung disease. We showed that five published CT scoring systems are comparable and have good intra- and interobserver agreement. Secondly, we showed that CT scoring systems and quantitative CT-measurements of airway wall thickening and bronchiectasis are more sensitive to detect the start and worsening of CF lung disease than are PFTs. Bronchiectasis-score worsened most in children and the worsening remained undetected by the PFTs and the quantitative measurements. Quantitative CT-measurements of air! way wall thickening worsened significantly, whereas PFTs and airway wall thickness measured by scoring remained unchanged. Thirdly, we developed a computational model to study radiation risks associated with CT scanning in CF. Risks from lifelong biennial CT scanning in CF were found to be acceptably low given the currently reduced life expectancy. Finally we provided normal CT-values of lung parenchyma and airway wall and lumen that can be used to study lung growth aberrations due to CF. Our data support routine CT scanning to monitor CF lung disease. In addition, bronchiectasis-score and quantitatively measured airway wall thickening may be useful surrogate endpoints for clinical trials in CF.