Radiography, tomosynthesis, CT and MRI in the evaluation of pulmonary cystic fibrosis: an untangling review of the multitude of scoring systems

Insights Imaging. 2013 Dec;4(6):787-98. doi: 10.1007/s13244-013-0288-y. Epub 2013 Sep 25.


Objective: The first radiographic scoring system for pulmonary cystic fibrosis was presented in 1958. Since then a multitude of scoring systems for radiography and computed tomography (CT) have been presented, recently also for tomosynthesis and magnetic resonance imaging (MRI). The aim of the current review was to analyse and compare the plethora of scoring systems for cystic fibrosis, especially regarding which scoring components are considered most important.

Methods: Four scoring systems for chest radiography, one for tomosynthesis, eight for CT and one for MRI were compared regarding components evaluated and their terminology; the areas scored; scoring levels; the weighting of each component in percentage of the total score; and the calculations for the final score.

Results: In most radiological scoring systems the lungs are evaluated for increased volume, bronchial wall thickening, bronchiectasis, mucus plugging, atelectasis and consolidation. In addition, for instance abscesses, bullae, septal thickening, mosaic perfusion, ground glass opacities and air trapping are evaluated in some CT scoring systems. Pleural affection and perfusion defects are scored on MRI.

Conclusions: Bronchiectasis alone, or in combination with mucus plugging, is given the highest weighting in most scoring systems and is thus commonly considered to be the most significant finding when evaluating cystic fibrosis lung disease.

Teaching points: • Scoring of examinations is used for comparison of outcome in studies. • Scoring of examinations can also be used for monitoring disease progression. • Cystic fibrosis can be scored on radiography, tomosynthesis, CT or MRI. • The typical imaging findings of cystic fibrosis depend on the imaging modality used. • Bronchiectasis is commonly considered the most significant finding when scoring cystic fibrosis.