Lung function tests are commonly used to monitor lung disease in cystic fibrosis (CF). While practical, they cannot locate the exact origin of functional impairment. Contemporary magnetic resonance imaging (MRI) techniques provide information on the location of disease but the need for contrast agents constrains their repeated application. We examined the correlation between functional MRI, performed without administration of contrast agent, and lung clearance index (LCI) from nitrogen multiple-breath washout (N2-MBW).40 children with CF (median (range) age 12.0 (6-18) years) and 12 healthy age-matched controls underwent functional and structural MRI and lung function tests on the same day. Functional MRI provided semiquantitative measures of perfusion (RQ) and ventilation (RFV) impairment as percentages of affected lung volume. Morphological MRI was evaluated using CF-specific scores. LCI measured global ventilation inhomogeneity.MRI detected functional impairment in CF: RFV 19-38% and RQ 16-35%. RFV and RQ correlated strongly with LCI (r=0.76, p<0.0001 and r=0.85, p<0.0001, respectively), as did total morphology score (r=0.81, p<0.0001). All indices differed significantly between patients with CF and healthy controls (p<0.001).Noninvasive functional MRI is a promising method to detect and visualise perfusion and ventilation impairment in CF without the need for contrast agents.
Copyright ©ERS 2017.