ABSTRACT
Progressive lung disease starts in most children with cystic brosis (CF) in infancy and progresses throughout life.1,2 e most important components of CF lung disease are bronchiectasis, which is an irreversible widening of bronchi, and trapped air which reects small airways disease.3,4 Imaging techniques that can be used to monitor CF lung disease in clinical trials are chest radiography, computed tomography (CT), and magnetic resonance imaging. Of these modalities CT is currently the most sensitive and feasible modality to diagnose and monitor bronchiectasis and trapped air in clinical trials. CT has been recognized as the gold standard for the detection of bronchiectasis since the mid-90s.5,6 Bronchiectasis as detected on inspiratory chest CT scans has been well validated as clinical outcome measure over the last decades.7,10 In more recent years, it has become clear that trapped air is also an early and important feature of CF lung disease. Trapped air can be detected on expiratory CT scans.11 Other structural ndings that can be observed on chest CT in CF and that are of interest in clinical trials are airway wall thickness and mucus impaction.12-14 It is now recognized that inclusion of chest CT-related outcome measures can be of importance in clinical trials as primary or secondary end point to determine the ecacy of an investigational drug treatment on progression of CF lung disease and or to characterize the patient population.15 To use chest CT in clinical trials, safe low-dose well-standardized protocols and reproducible sensitive image analysis of the structural changes of interest are needed. e aim of this chapter is to describe the importance of chest CT-related outcome measures, how CTs can be used in clinical trials, and how images can be analyzed.
