Ten years of chest MRI for patients with cystic fibrosis : Translation from the bench to clinical routine.

Abstract

Despite recent advances in our knowledge about the pathophysiology and treatment of cystic fibrosis (CF), pulmonary involvement remains the most important determinant of morbidity and mortality in patients with CF. Since lung function testing may not be sensitive enough for subclinical disease progression, and because young children may have normal spirometry results over alonger period of time, imaging today plays an increasingly important role in clinical routine and research for the monitoring of CF lung disease. In this regard, chest magnetic resonance imaging (MRI) could serve as aradiation-free modality for structural and functional lung imaging. Our research agenda encompassed the entire process of development, implementation, and validation of appropriate chest MRI protocols for use with infant and adult CF patients alike. After establishing ageneral MRI protocol for state-of-the-art clinical 1.5-T scanners based on the available sequence technology, asemiquantitative scoring system was developed followed by cross-validation of the method against the established modalities of computed tomography, radiography, and lung function testing. Cross-sectional studies were then set up to determine the sensitivity of the method for the interindividual variation of the disease and for changes in disease severity after treatment. Finally, the MRI protocol was implemented at multiple sites to be validated in amulticenter setting. After more than adecade, lung MRI has become avaluable tool for monitoring CF in clinical routine application and as an endpoint for clinical studies.