Imaging modalities in cystic fibrosis: emerging role of MRI

MRI has emerged as radiation-free imaging modality for assessment of cystic fibrosis lung disease. Here, we review the current status and new developments of MRI in cystic fibrosis.

MRI is sensitive to detect abnormalities in lung structure and perfusion, even in preschool children with cystic fibrosis with early lung disease. Further, MRI is sensitive to detect changes associated with pulmonary exacerbations and response to antibiotic therapy in this challenging age group.

MRI is sensitive to detect hallmarks of cystic fibrosis lung disease such as bronchial wall thickening, bronchiectasis, mucus plugging and abnormal lung perfusion. A morpho-functional MRI score has been established for semiquantitative assessment of these characteristic abnormalities over a broad range of disease severity. Recent studies demonstrated that MRI is sensitive to detect changes in mucus plugging and lung perfusion in response to antibiotic therapy for pulmonary exacerbations. These results suggest that MRI may be suitable for noninvasive monitoring and as a quantitative endpoint in clinical trials for cystic fibrosis. Technical advances including ultra-short echo time and Fourier decomposition imaging are expected to enhance morphological and functional MRI of cystic fibrosis lung disease without the need of contrast medium in the near future.