{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,23]],"date-time":"2026-10-23T06:02:07Z","timestamp":1792735327106,"version":"4.5.0"},"reference-count":34,"publisher":"Oxford University Press (OUP)","issue":"6","license":[{"start":{"date-parts":[[2026,2,3]],"date-time":"2026-02-03T00:00:00Z","timestamp":1770076800000},"content-version":"vor","delay-in-days":1,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/100000897","name":"Cystic Fibrosis Foundation","doi-asserted-by":"publisher","award":["MCNALL19K0"],"award-info":[{"award-number":["MCNALL19K0"]}],"id":[{"id":"10.13039\/100000897","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100000292","name":"Cystic Fibrosis Trust","doi-asserted-by":"publisher","award":["VIA083"],"award-info":[{"award-number":["VIA083"]}],"id":[{"id":"10.13039\/501100000292","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100023551","name":"Cystic Fibrosis Ireland","doi-asserted-by":"publisher","award":["RECOVER01"],"award-info":[{"award-number":["RECOVER01"]}],"id":[{"id":"10.13039\/501100023551","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2026,6,1]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Rationale<\/jats:title>\n                    <jats:p>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.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Objectives<\/jats:title>\n                    <jats:p>Using multicenter standardized image collection and automated analysis, we sought to establish whether improvements in SLD with ETI continued in the second year of treatment.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>Spirometry-controlled CT scans were performed at 6 sites in people with CF aged \u226512 years homozygous for the F508del mutation or heterozygous for the F508del and a minimum function mutation at baseline and 12\u2009months and 24\u2009months after commencing ETI. CT scans were analyzed using the automated LungQ platform (Thirona) to measure mucus plugging, trapped air, and bronchus-artery (BA) pair measurements: bronchial outer diameter (Bout), bronchial inner diameter (Bin), bronchial wall thickness (Bwt), arterial diameter (A), and BA ratios: Bout\/A, Bin\/A, Bwt\/A, and bronchial wall area\/bronchial outer area (Bwa\/Boa). Scans were also visually scored using the PRAGMA-CF scoring system, divided into % disease (%DIS), % bronchiectasis (%BX), % mucus plugging (%MP), % bronchial wall thickening (%BWT), and % trapped air (%TA).<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>CT scans were performed on 79 participants at baseline, 64 at 12\u2009months, and 52 at 24\u2009months. Automated analysis showed a significant reduction BwtA, and Bwa\/Boa at 12\u2009months, which were sustained to 24\u2009months. No change was seen in Bin\/A or Bout\/A at 12 or 24\u2009months. Improvements in mucus plug numbers, plug volume, and %TA at 12\u2009months were sustained at 24\u2009months. ETI was associated with a reduction in the ratio of pulmonary blood volume in the arterial system compared to the venous system in vessels of &amp;lt;1 mm, 1-2 mm, and &amp;gt;2 mm diameter. Manual PRAGMA-CF scores demonstrated improvements in %DIS, %BX, %MP, %BWT, and %TA at 12\u2009months, and these changes were sustained, unchanged to 24\u2009months.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>ETI is associated with substantial improvements in bronchial wall thickening, mucus plugging, and trapped air at 12\u2009months. Changes were maintained to 24\u2009months and did not continue to improve. Abnormal bronchial widening remained stable and did not improve with ETI therapy. Changes in the distribution of pulmonary arterial\/venous blood volumes with ETI suggest a beneficial impact on pulmonary vascular pressures.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1093\/annalsats\/aaoag021","type":"journal-article","created":{"date-parts":[[2026,2,3]],"date-time":"2026-02-03T04:50:53Z","timestamp":1770094253000},"page":"894-907","source":"Crossref","is-referenced-by-count":3,"title":["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"],"prefix":"10.1093","volume":"23","author":[{"given":"Paul","family":"McNally","sequence":"first","affiliation":[{"name":"Department of Paediatrics, RCSI University of Medicine and Health Sciences , Dublin,","place":["Ireland"]},{"name":"Departments of Respiratory Medicine and Radiology, Children\u2019s Health Ireland , 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Dublin,","place":["Ireland"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mike","family":"Williamson","sequence":"additional","affiliation":[{"name":"Departments of Respiratory Medicine and Radiology, Children\u2019s Health Ireland , Dublin,","place":["Ireland"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Des W","family":"Cox","sequence":"additional","affiliation":[{"name":"Departments of Respiratory Medicine and Radiology, Children\u2019s Health Ireland , Dublin,","place":["Ireland"]},{"name":"School of Medicine, University College Dublin , Dublin,","place":["Ireland"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Barry","family":"Linnane","sequence":"additional","affiliation":[{"name":"School of Medicine, University of Limerick , Limerick,","place":["Ireland"]},{"name":"Department of Paediatrics and Radiology, Limerick University Hospital , Limerick,","place":["Ireland"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Siobhan","family":"McGrane","sequence":"additional","affiliation":[{"name":"School of Medicine, University of Limerick , Limerick,","place":["Ireland"]},{"name":"Department of Paediatrics and Radiology, Limerick University Hospital , Limerick,","place":["Ireland"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Laura","family":"Kirwan","sequence":"additional","affiliation":[{"name":"Cystic Fibrosis Registry of Ireland , Dublin,","place":["Ireland"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Paul","family":"O\u2019Regan","sequence":"additional","affiliation":[{"name":"Cystic Fibrosis Registry of Ireland , Dublin,","place":["Ireland"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Merlijn","family":"Bonte","sequence":"additional","affiliation":[{"name":"Departments of Paediatric Respiratory Medicine and Radiology, Erasmus Medical Centre , 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Dublin,","place":["Ireland"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5455-8208","authenticated-orcid":false,"given":"Edward F","family":"McKone","sequence":"additional","affiliation":[{"name":"School of Medicine, University College Dublin , Dublin,","place":["Ireland"]},{"name":"Departments of Respiratory Medicine and Radiology, St Vincent\u2019s University Hospital , Dublin,","place":["Ireland"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Clare","family":"Saunders","sequence":"additional","affiliation":[{"name":"Department of Paediatric Respiratory Medicine, Royal Brompton & Harefield Hospitals , London,","place":["United Kingdom"]},{"name":"National Heart and Lung Institute, Imperial College London , London,","place":["United Kingdom"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Thomas","family":"Semple","sequence":"additional","affiliation":[{"name":"Department of Paediatric Respiratory Medicine, Royal Brompton & Harefield Hospitals , London,","place":["United Kingdom"]},{"name":"National Heart and Lung Institute, Imperial College London , London,","place":["United Kingdom"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jane C","family":"Davies","sequence":"additional","affiliation":[{"name":"Department of Paediatric Respiratory Medicine, Royal Brompton & Harefield Hospitals , London,","place":["United Kingdom"]},{"name":"National Heart and Lung Institute, Imperial College London , London,","place":["United Kingdom"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Harm A W M","family":"Tiddens","sequence":"additional","affiliation":[{"name":"Departments of Paediatric Respiratory Medicine and Radiology, Erasmus Medical Centre , Rotterdam,","place":["Netherlands"]},{"name":"Thirona , Nijmegen,","place":["Netherlands"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"name":"the RECOVER Study 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