{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,1]],"date-time":"2026-10-01T12:38:54Z","timestamp":1790858334732,"version":"4.1.0"},"reference-count":36,"publisher":"European Respiratory Society (ERS)","issue":"3","license":[{"start":{"date-parts":[[2024,2,8]],"date-time":"2024-02-08T00:00:00Z","timestamp":1707350400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/www.ersjournals.com\/user-licence"}],"funder":[{"name":"Fili\u00e8re maladies rares Muco CFTR"},{"name":"Soci\u00e9t\u00e9 Fran\u00e7aise de la Mucoviscidose"},{"DOI":"10.13039\/501100006342","name":"Association Vaincre la Mucoviscidose","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100006342","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["publications.ersnet.org"],"crossmark-restriction":true},"short-container-title":["Eur Respir J"],"accepted":{"date-parts":[[2024,1,30]]},"published-print":{"date-parts":[[2024,3]]},"abstract":"<jats:sec><jats:title>Background<\/jats:title><jats:p>This study sought to evaluate the impact of elexacaftor\/tezacaftor\/ivacaftor (ETI) on lung structural abnormalities in adults with cystic fibrosis (awCF) with a specific focus on the reversal of bronchial dilatations.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Chest computed tomography (CT) scans performed prior to and 12\u2005months after initiation of ETI were visually reviewed for possible reversal of bronchial dilatations. AwCF with and without reversal of bronchial dilatations (the latter served as controls, with three controls per case) were selected. Visual Brody score, bronchial and arterial diameters, and lung volume were measured on CT.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Reversal of bronchial dilatations was found in 12\/235 (5%) awCF treated with ETI. 12 awCF with and 36 without reversal of bronchial dilatations were further analysed (male 56%, mean\u00b1<jats:sc>sd<\/jats:sc>age 31.6\u00b18.5\u2005years, F508del\/F508del<jats:italic>CFTR<\/jats:italic>54% and mean forced expiratory volume in 1\u2005s 58.8\u00b122.3% predicted). The Brody score improved overall from 79.4\u00b129.8 to 54.8\u00b132.3 (p&lt;0.001). Reversal of bronchial dilatations was confirmed by a decrease in bronchial lumen diameter in cases from 3.9\u00b10.9 to 3.2\u00b11.1\u2005mm (p&lt;0.001), whereas it increased in awCF without reversal of bronchial dilatations (from 3.5\u00b11.1 to 3.6\u00b11.2\u2005mm; p=0.002). Reversal of bronchial dilatations occurred in cylindrical (not varicose or saccular) bronchial dilatations. Lung volumes decreased by \u22126.6\u00b110.7% in awCF with reversal of bronchial dilatations but increased by +2.3\u00b19.6% in controls (p=0.007).<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>Although bronchial dilatations are generally considered irreversible, ETI was associated with reversal, which was limited to the cylindrical bronchial dilatation subtype, and occurred in a small subset of awCF. Initiating ETI earlier in life may reverse early bronchial dilatations.<\/jats:p><\/jats:sec>","DOI":"10.1183\/13993003.01794-2023","type":"journal-article","created":{"date-parts":[[2024,2,8]],"date-time":"2024-02-08T16:45:28Z","timestamp":1707410728000},"page":"2301794","update-policy":"https:\/\/doi.org\/10.1183\/ers-crossmark-policy","source":"Crossref","is-referenced-by-count":24,"title":["Reversal of cylindrical bronchial dilatations in a subset of adults with cystic fibrosis treated with elexacaftor\/tezacaftor\/ivacaftor"],"prefix":"10.1183","volume":"63","author":[{"given":"Paul","family":"Cazier","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Guillaume","family":"Chassagnon","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Th\u00e9o","family":"Dhote","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jennifer","family":"Da Silva","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Reem","family":"Kanaan","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Isabelle","family":"Honor\u00e9","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6828-608X","authenticated-orcid":false,"given":"Nicolas","family":"Carlier","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8213-7817","authenticated-orcid":false,"given":"Marie-Pierre","family":"Revel","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Emma","family":"Canniff","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4385-5888","authenticated-orcid":false,"given":"Cl\u00e9mence","family":"Martin","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0903-9828","authenticated-orcid":false,"given":"Pierre-R\u00e9gis","family":"Burgel","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"81","published-online":{"date-parts":[[2024,2,8]]},"reference":[{"key":"2024102103225078000_63.3.2301794.1","doi-asserted-by":"crossref","first-page":"65","DOI":"10.1016\/S2213-2600(19)30337-6","article-title":"The future of cystic fibrosis care: a global perspective","volume":"8","author":"Bell","year":"2020","journal-title":"Lancet Respir Med"},{"key":"2024102103225078000_63.3.2301794.2","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(20)32542-3"},{"key":"2024102103225078000_63.3.2301794.3","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1105185"},{"key":"2024102103225078000_63.3.2301794.4","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.202011-4153OC"},{"key":"2024102103225078000_63.3.2301794.5","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(19)32597-8"},{"key":"2024102103225078000_63.3.2301794.6","doi-asserted-by":"crossref","first-page":"188","DOI":"10.1164\/rccm.201906-1227OC","article-title":"Real-life safety and effectiveness of lumacaftor\u2013ivacaftor in patients with cystic fibrosis","volume":"201","author":"Burgel","year":"2020","journal-title":"Am J Respir Crit Care Med"},{"key":"2024102103225078000_63.3.2301794.7","doi-asserted-by":"crossref","first-page":"529","DOI":"10.1164\/rccm.202108-1986OC","article-title":"Clinical effectiveness of elexacaftor\/tezacaftor\/ivacaftor in people with cystic fibrosis: a clinical trial","volume":"205","author":"Nichols","year":"2022","journal-title":"Am J Respir Crit Care Med"},{"key":"2024102103225078000_63.3.2301794.8","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1908639"},{"key":"2024102103225078000_63.3.2301794.9","doi-asserted-by":"crossref","first-page":"e122695","DOI":"10.1172\/jci.insight.122695","article-title":"Effect of ivacaftor on mucociliary clearance and clinical outcomes in cystic fibrosis patients with G551D-CFTR","volume":"3","author":"Donaldson","year":"2018","journal-title":"JCI Insight"},{"key":"2024102103225078000_63.3.2301794.10","doi-asserted-by":"publisher","DOI":"10.1016\/j.jcf.2014.06.011"},{"key":"2024102103225078000_63.3.2301794.11","doi-asserted-by":"crossref","first-page":"248","DOI":"10.1183\/13993003.01918-2015","article-title":"Long-term computed tomographic changes in cystic fibrosis patients treated with ivacaftor","volume":"48","author":"Chassagnon","year":"2016","journal-title":"Eur Respir J"},{"key":"2024102103225078000_63.3.2301794.12","doi-asserted-by":"crossref","first-page":"1999","DOI":"10.3390\/jcm10091999","article-title":"Computed tomographic changes in patients with cystic fibrosis treated by combination therapy with lumacaftor and ivacaftor","volume":"10","author":"Arnaud","year":"2021","journal-title":"J Clin Med"},{"key":"2024102103225078000_63.3.2301794.13","doi-asserted-by":"crossref","first-page":"2101344","DOI":"10.1183\/13993003.01344-2021","article-title":"Using chest computed tomography and unsupervised machine learning for predicting and evaluating response to lumacaftor\u2013ivacaftor in people with cystic fibrosis","volume":"59","author":"Campredon","year":"2021","journal-title":"Eur Respir J"},{"key":"2024102103225078000_63.3.2301794.14","doi-asserted-by":"crossref","first-page":"1147348","DOI":"10.3389\/fphar.2023.1147348","article-title":"The effect of CFTR modulators on structural lung disease in cystic fibrosis","volume":"14","author":"Mok","year":"2023","journal-title":"Front Pharmacol"},{"key":"2024102103225078000_63.3.2301794.15","doi-asserted-by":"crossref","first-page":"1053","DOI":"10.1016\/j.jcf.2022.03.011","article-title":"Magnetic resonance imaging detects improvements of pulmonary and paranasal sinus abnormalities in response to elexacaftor\/tezacaftor\/ivacaftor therapy in adults with cystic fibrosis","volume":"21","author":"Wucherpfennig","year":"2022","journal-title":"J Cyst Fibros"},{"key":"2024102103225078000_63.3.2301794.16","doi-asserted-by":"crossref","first-page":"298","DOI":"10.1016\/S2213-2600(21)00277-0","article-title":"Criteria and definitions for the radiological and clinical diagnosis of bronchiectasis in adults for use in clinical trials: international consensus recommendations","volume":"10","author":"Aliberti","year":"2022","journal-title":"Lancet Respir Med"},{"key":"2024102103225078000_63.3.2301794.17","doi-asserted-by":"publisher","DOI":"10.1016\/j.jpeds.2003.11.008"},{"key":"2024102103225078000_63.3.2301794.18","doi-asserted-by":"crossref","first-page":"1011","DOI":"10.1148\/rg.2015140214","article-title":"Bronchiectasis: mechanisms and imaging clues of associated common and uncommon diseases","volume":"35","author":"Milliron","year":"2015","journal-title":"Radiographics"},{"key":"2024102103225078000_63.3.2301794.19","doi-asserted-by":"crossref","first-page":"1068103","DOI":"10.3389\/fped.2023.1068103","article-title":"MDCT-based longitudinal automated airway and air trapping analysis in school-age children with mild cystic fibrosis lung disease","volume":"11","author":"Weinheimer","year":"2023","journal-title":"Front Pediatr"},{"key":"2024102103225078000_63.3.2301794.20","doi-asserted-by":"crossref","first-page":"311","DOI":"10.1164\/rccm.202201-0219OC","article-title":"Effects of elexacaftor\/tezacaftor\/ivacaftor therapy on lung clearance index and magnetic resonance imaging in patients with cystic fibrosis and one or two F508del alleles","volume":"206","author":"Graeber","year":"2022","journal-title":"Am J Respir Crit Care Med"},{"key":"2024102103225078000_63.3.2301794.21","doi-asserted-by":"crossref","first-page":"917","DOI":"10.1164\/rccm.202308-1317OC","article-title":"Improvement in lung clearance index and chest computed tomography scores with elexacaftor\/tezacaftor\/ivacaftor treatment in people with cystic fibrosis aged 12\u2005years and older \u2013 the RECOVER trial","volume":"208","author":"McNally","year":"2023","journal-title":"Am J Respir Crit Care Med"},{"key":"2024102103225078000_63.3.2301794.22","doi-asserted-by":"crossref","first-page":"110421","DOI":"10.1016\/j.ejrad.2022.110421","article-title":"Chest computed tomography improvement in patients with cystic fibrosis treated with elexacaftor-tezacaftor-ivacaftor: early report","volume":"154","author":"Bec","year":"2022","journal-title":"Eur J Radiol"},{"key":"2024102103225078000_63.3.2301794.23","doi-asserted-by":"crossref","first-page":"63","DOI":"10.1590\/S0041-87812001000300001","article-title":"Persistent asthma in adults: comparison of high resolution computed tomography of the lungs after one year of follow-up","volume":"56","author":"Cukier","year":"2001","journal-title":"Rev Hosp Clin Fac Med Sao Paulo"},{"key":"2024102103225078000_63.3.2301794.24","doi-asserted-by":"publisher","DOI":"10.1016\/S0720-048X(02)00122-5"},{"key":"2024102103225078000_63.3.2301794.25","doi-asserted-by":"publisher","DOI":"10.1136\/thx.2003.011577"},{"key":"2024102103225078000_63.3.2301794.26","first-page":"514","article-title":"Efficacy of intravenous immunoglobulin treatment in children with common variable immunodeficiency","volume":"21","author":"Baris","year":"2011","journal-title":"J Investig Allergol Clin Immunol"},{"key":"2024102103225078000_63.3.2301794.27","doi-asserted-by":"crossref","first-page":"966","DOI":"10.1111\/1440-1681.13503","article-title":"Reversible bronchial dilatation in adults","volume":"48","author":"Zhang","year":"2021","journal-title":"Clin Exp Pharmacol Physiol"},{"key":"2024102103225078000_63.3.2301794.28","doi-asserted-by":"crossref","first-page":"S38","DOI":"10.1016\/S1569-1993(15)30120-X","article-title":"WS20.5 Cystic fibrosis related structural lung disease on high resolution computed tomography before and after ivacaftor therapy","volume":"14","author":"Sayers","year":"2015","journal-title":"J Cyst Fibros"},{"key":"2024102103225078000_63.3.2301794.29","doi-asserted-by":"publisher","DOI":"10.1016\/j.chest.2017.10.005"},{"key":"2024102103225078000_63.3.2301794.30","doi-asserted-by":"publisher","DOI":"10.1136\/thoraxjnl-2011-200912"},{"key":"2024102103225078000_63.3.2301794.31","doi-asserted-by":"publisher","DOI":"10.1002\/path.2118"},{"key":"2024102103225078000_63.3.2301794.32","doi-asserted-by":"publisher","DOI":"10.1183\/09031936.00221711"},{"key":"2024102103225078000_63.3.2301794.33","first-page":"535","article-title":"Regional distribution of macroscopic lung disease in cystic fibrosis","volume":"133","author":"Tomashefski","year":"1986","journal-title":"Am Rev Respir Dis"},{"key":"2024102103225078000_63.3.2301794.34","doi-asserted-by":"publisher","DOI":"10.1148\/radiol.2532090306"},{"key":"2024102103225078000_63.3.2301794.35","doi-asserted-by":"publisher","DOI":"10.1183\/13993003.02153-2022"},{"key":"2024102103225078000_63.3.2301794.36","doi-asserted-by":"publisher","DOI":"10.1136\/thorax-2022-219943"}],"container-title":["European Respiratory Journal"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/syndication.highwire.org\/content\/doi\/10.1183\/13993003.01794-2023","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,2,24]],"date-time":"2025-02-24T07:17:53Z","timestamp":1740381473000},"score":1,"resource":{"primary":{"URL":"https:\/\/publications.ersnet.org\/lookup\/doi\/10.1183\/13993003.01794-2023"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2024,2,8]]},"references-count":36,"journal-issue":{"issue":"3","published-online":{"date-parts":[[2024,3,28]]},"published-print":{"date-parts":[[2024,3]]}},"alternative-id":["10.1183\/13993003.01794-2023"],"URL":"https:\/\/doi.org\/10.1183\/13993003.01794-2023","relation":{},"ISSN":["0903-1936","1399-3003"],"issn-type":[{"value":"0903-1936","type":"print"},{"value":"1399-3003","type":"electronic"}],"subject":[],"published":{"date-parts":[[2024,2,8]]}}}