Magnetic resonance imaging of the lungs in cystic fibrosis

Abstract

Disease severity and mortality in patients with cystic fibrosis (CF) is mainly determined by (progressive) pulmonary lung disease. Early diagnosis and therapy are important and of prognostic value to conserve lung function. Primary imaging techniques for lung imaging are x‑ray and computed tomography (CT) to monitor disease severity and regional distribution. Radiation-free imaging techniques such as magnetic resonance imaging (MRI) have gained interest over the last decade in order to prevent radiation damage. The main findings of CF lung disease are airway wall thickening, bronchiectasis, and mucus plugging, which are found in up to 60% of preschool age children. Pleural abnormalities and consolidations are often associated with pulmonary exacerbation. Young CF patients often show amosaic pattern as functional changes and also perfusion defects can be seen from birth in 50% of CF patients by contrast-enhanced perfusion imaging, and in up to 90% of adult patients, with varying degrees of severity. Dilated bronchial arteries indicate an increased risk for hemoptysis. Proton MRI is the sole imaging technique that can show structural and functional lung changes in one examination. Structured assessment using ascoring system helps to systematically grade the extent and severity of all CF-associated changes. Lung MRI for cystic fibrosis has been recently established as aclinical standard examination and is routinely performed at experienced centers. More recently, it has also been used as an endpoint within the framework of clinical studies.