Early Effects of Elexacaftor-Tezacaftor-Ivacaftor Therapy on Magnetic Resonance Imaging in Patients with Cystic Fibrosis and Advanced Lung Disease

This is a prospective, observational study involving three Cystic Fibrosis (CF) adult patients, evaluating the changes in chest magnetic resonance imaging (MRI) three months after the start of elexacaftor/tezacaftor and ivacaftor therapy. MRI showed a drastic reduction in mucus plugging and bronchial wall thickening, with an improvement in the diffusion-weighted MRI score. Similarly, a marked improvement in spirometric parameters, nutritional status, and sweat chloride was observed. Our preliminary data confirm that chest MRI could be a useful tool to assess disease progression in CF patients on modulatory drug therapy.

Automated summary

This observational study examined the changes in chest MRI of three CF adult patients three months after starting elexacaftor/tezacaftor and ivacaftor therapy. The results showed a significant reduction in mucus plugging and bronchial wall thickening, as well as an improvement in diffusion-weighted MRI score. Spirometric parameters, nutritional status, and sweat chloride also improved. These preliminary findings suggest that chest MRI could be a valuable tool for assessing disease progression in CF patients receiving modulatory drug therapy.