Impact of 2 years of treatment with elexacaftor/tezacaftor/ivacaftor on longitudinal changes in structural lung disease in people with cystic fibrosis: results from the RECOVER trial.

Paul McNally^1,2, Karen Lester^1,2, Rachel Cregan^1,2, Basil Elnazir^2,3, Thara Persaud², David Rea², Eilish Twomey², Mike Williamson², Des W Cox^2,4, Barry Linnane^5,6, Siobhan McGrane^5,6, Laura Kirwan⁷, Paul O'Regan⁷, Merlijn Bonte⁸, Punit Makani⁸, Daan Caudri⁸, Jonathan D Dodd^4,9, Edward F McKone^4,9, Clare Saunders^10,11, Thomas Semple^10,11, Jane C Davies^10,11, Harm A W M Tiddens^8,12,

¹Department of Paediatrics, RCSI University of Medicine and Health Sciences, Dublin, Ireland.

²Departments of Respiratory Medicine and Radiology, Children's Health Ireland, Dublin, Ireland.

³School of Medicine, Trinity College, Dublin, Ireland.

⁴School of Medicine, University College Dublin, Dublin, Ireland.

⁵School of Medicine, University of Limerick, Limerick, Ireland.

⁶Department of Paediatrics and Radiology, Limerick University Hospital, Limerick, Ireland.

⁷Cystic Fibrosis Registry of Ireland, Dublin, Ireland.

⁸Departments of Paediatric Respiratory Medicine and Radiology, Erasmus Medical Centre, Rotterdam, Netherlands.

⁹Departments of Respiratory Medicine and Radiology, St Vincent's University Hospital, Dublin, Ireland.

¹⁰Department of Paediatric Respiratory Medicine, Royal Brompton & Harefield Hospitals, London, United Kingdom.

¹¹National Heart and Lung Institute, Imperial College London, London, United Kingdom.

¹²Thirona, Nijmegen, Netherlands.

PubMed PMC DOI PDF

Abstract

RATIONALE: Progressive structural lung disease (SLD) is a key hallmark of cystic fibrosis (CF). Elexacaftor/tezacaftor/ivacaftor (ETI) is associated with short-term improvements in SLD measured on chest computed tomography (CT). Longer-term changes in SLD outcomes with ETI are unknown. OBJECTIVES: Using multicenter standardized image collection and automated analysis, we sought to establish wheth…

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Mentioned Companies

Thirona
affiliation

Other Funding

Cystic Fibrosis Foundation(MCNALL19K0)
Cystic Fibrosis Trust(VIA083)
Cystic Fibrosis Ireland(RECOVER01)

MeSH Terms

Adolescent0Adult0Child0Female0Humans0Male0Young Adult0Aminophenols*Benzodioxoles*Chloride Channel Agonists0Cystic Fibrosis*Disease Progression0Drug Combinations0Indoles*Lung*Pyrazoles*Pyridines*Pyrrolidines*Quinolones*Spirometry0Tomography, X-Ray Computed0Treatment Outcome0Quinolines0

Keywords

Automated scoringBronchiectasisComputerised TomographyElexacaftor/Tezacaftor/Ivacaftorcystic fibrosis

Article Info

PMID: 41973991
PMCID: PMC13235859
DOI: 10.1093/annalsats/aaoag021
Received: August 11, 2025
Accepted: December 19, 2025

How to Cite

McNally P et al. Impact of 2 years of treatment with elexacaftor/tezacaftor/ivacaftor on longitudinal changes in structural lung disease in people with cystic fibrosis: results from the RECOVER trial. Ann Am Thorac Soc2026;23(6):894-907. doi:10.1093/annalsats/aaoag021

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