Chest radiographic findings in cystic fibrosis

Radiographic imaging modalities that have been applied to the staging and prognostication of the lung lesion in cystic fibrosis (CF) include conventional chest radiographs, computed tomography (CT), and magnetic resonance imaging (MRI). Conventional chest radiographs are usually adequate to detect the salient radiographic features of CF and provide objective parameters for longitudinal disease progression. Although the lung manifestations of CF can be highly variable most patients with CF demonstrate some of the classic chest radiographic findings that reflect chronic bronchiectasis: hyperinflation, bronchial thickening and dilatation, peribronchial cuffing, mucoid impaction, cystic radiolucencies, an increase in interstitial markings, and scattered nodular densities. Complications of advanced CF include atelectasis, mucoid impaction, pneumothorax, pneumomediastinum, pulmonary hemorrhage, cardiomegaly, and enlargement of the pulmonary artery with cor pulmonale. Scoring systems have been developed to grade the chest radiograph in CF and to more accurately quantitate the severity of disease. Currently, the Brasfield system is in widest use. These radiographic scoring systems correlate well with pulmonary function data in both the pediatric and adult CF populations. High-resolution computed tomography (HRCT) is clearly more sensitive and accurate than conventional chest radiography in delineating the extent and severity of bronchiectasis and other parenchymal and airway lesions in CF. Quantifiable scoring systems using HRCT have been developed to assess disease severity in CF. The use of MRI in the clinical management of CF has been limited. MRI may be helpful in determining the cause of linear lung markings, differentiating mucous plugging, and peribronchial thickening from normal pulmonary blood vessels. MRI is an excellent imaging modality to differentiate hilar or mediastinal adenopathy from blood vessels or mediastinal fat.