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.


 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.


 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.
C
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).



Elexacaftor/Tezacaftor/Ivacaftor
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).


 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.


 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).



MRI
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. 
MR angiography
MRI was assessed using the validated chest MRI score, and presence and total lumen area of BAD were assessed with commercial software.



commercial software