Elexacaftor/Tezacaftor/Ivacaftor Improves Bronchial Artery Dilatation Detected by Magnetic Resonance Imaging in Patients with Cystic Fibrosis

RATIONALE: Magnetic resonance imaging (MRI) detects improvements in mucus plugging and bronchial wall thickening, but not in lung perfusion in patients with cystic fibrosis (CF) treated with elexacaftor/tezacaftor/ivacaftor (ETI). OBJECTIVE(S): To determine whether bronchial artery dilatation (BAD), a key feature of advanced lung disease, indicates irreversibility of perfusion abnormalities and whether BAD could be reversed in CF patients treated with ETI. METHODS: 59 adults with CF underwent longitudinal chest MRI including MR angiography (MRA) twice, 35 CF patients (mean age 31±7y) before (MRI1) and after (MRI2) at least one month (mean duration 8±4mon) on ETI therapy and 24 control CF patients (mean age 31±7y) without ETI. MRI was assessed using the validated chest MRI score, and presence and total lumen area of BAD were assessed with commercial software. RESULTS: The MRI global score was stable in the control group from MRI1 to MRI 2 (mean difference: 1.1 [-0.3, 2.4], P=0.054), but was reduced in the ETI group (-10.1 [-0.3, 2.4], P<0.001). In the control as well as in the ETI group, BAD was present in almost all patients at baseline (95% and 94%, respectively) which did not change at MRI2. The BAD total lumen area did not change in the control group from MRI1 to MRI2 (1.0 mm2 [-0.2, 2.2], P=0.099), but decreased in the ETI group (-7.0 mm2 [-8.9, -5.0], P<0.001). This decrease correlated with improvements in the MRI global score (r=0.540, P<0.001). CONCLUSIONS: Our data show that BAD may be partially reversible under ETI therapy in adult CF patients with established disease.

• Wucherpfennig, L.
• Triphan, S. M. F.
• Wege, S.
• Kauczor, H. U.
• Heussel, C. P.
• Sommerburg, O.
• Stahl, M.
• Mall, M. A.
• Eichinger, M.
• Wielpütz, M. O.