Cystic fibrosis (CF) is a genetic disease that affects young people, with 90% mortality due to lung disease at a median age of approximately 35 years, despite significant improvements in the management of the disease. The greatest impediment to the evaluation of the many emerging therapies is the lack of adequate outcome surrogates for clinically meaningful endpoints. Magnetic resonance imaging (MRI) can evaluate both lung structure and function, does not use ionizing radiation, and has shown promising early results applied to CF. The purpose of this exploratory pilot study is to determine the ability of MRI to discriminate CF disease severity compared with existing clinical standards, including chest radiograph (CXR) and Forced Expiratory Volume in 1 second (FEV1), and existing research standards including computed tomography (CT) and the Cystic Fibrosis Questionnaire (CFQ), a disease-specific quality-of-life measurement tool. The results of this study will provide the information necessary to design an abbreviated MRI protocol for use in larger prospective randomized drug trials of therapeutic effectiveness.
January 2013 to February 2018