Dynamic Perfluorinated Gas MRI Shows Improved Lung Ventilation in People with Cystic Fibrosis after Elexacaftor/Tezacaftor/Ivacaftor: An Observational Study

The availability of highly effective CFTR modulators is revolutionizing the treatment of cystic fibrosis (CF) and drastically improving outcomes. MRI-based imaging modalities are now emerging as highly sensitive endpoints, particularly in the setting of mild lung disease. Adult CF patients were recruited from a single center prior to starting treatment with E/T/I. The following studies were obtained before and after one month on treatment: spirometry, multiple breath nitrogen washout (MBW), H-1 UTE MRI (structural images) and F-19 MRI (ventilation images). Changes between visits were calculated, as were correlations between FEV1, lung clearance index (LCI), MRI structural scores, and MRI-based ventilation descriptors. Eight subjects had complete datasets for evaluation. Consistent with prior clinical trials, FEV1 and LCI improved after 28 days of E/T/I use. H-1 UTE MRI detected improvements in bronchiectasis/airway wall thickening score and mucus plugging score after 28 days of therapy. F-19 MRI demonstrated improvements in fractional lung volume with slow gas washout time (FLV up arrow tau2) and ventilation defect percentage (VDP). Improvements in FLV up arrow tau2 and VDP correlated with improvement in FEV1 (r = 0.81 and 0.86, respectively, p < 0.05). This observational study establishes the ability of F-19 MRI and H-1 UTE MRI to detect improvements in lung structure and function after E/T/I treatment. This study supports further development of F-19 MRI and H-1 UTE MRI as outcome measures for cystic fibrosis research and drug development.

Automated summary

The availability of highly effective CFTR modulators is revolutionizing the treatment of cystic fibrosis, and MRI-based imaging modalities are emerging as highly sensitive endpoints in monitoring the effectiveness of the treatment. This observational study demonstrates the ability of F-19 MRI and H-1 UTE MRI to detect improvements in lung structure and function after E/T/I treatment, further supporting their potential as outcome measures for cystic fibrosis research and drug development.