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Impact of two years of treatment with Elexacaftor/Tezacaftor/Ivacaftor on longitudinal changes in structural lung disease in people with Cystic Fibrosis - Results from the RECOVER trial - PubMed

3 hours ago
  • #ETI Therapy
  • #Cystic Fibrosis
  • #CT Scan
  • The RECOVER trial assessed the impact of two years of Elexacaftor/Tezacaftor/Ivacaftor (ETI) treatment on structural lung disease (SLD) in people with cystic fibrosis (CF) aged 12+ using spirometry-controlled CT scans.
  • Automated analysis via LungQ® showed significant and sustained reductions at 24 months in bronchial wall thickness (Bwt/A), bronchial wall area/outer area (Bwa/Boa), mucus plugging, and trapped air, with no further improvement after 12 months.
  • Bronchial widening (Bout/A and Bin/A) remained stable and did not improve with ETI therapy.
  • ETI altered pulmonary blood volume distribution, reducing arterial-to-venous ratios across vessel sizes, suggesting beneficial effects on pulmonary vascular pressures.
  • Manual PRAGMA-CF scores confirmed improvements in disease extent, bronchiectasis, mucus plugging, bronchial wall thickening, and trapped air at 12 months, sustained at 24 months.