{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,1]],"date-time":"2026-10-01T06:14:47Z","timestamp":1790835287413,"version":"4.1.0"},"reference-count":39,"publisher":"European Respiratory Society (ERS)","issue":"6","license":[{"start":{"date-parts":[[2024,6,20]],"date-time":"2024-06-20T00:00:00Z","timestamp":1718841600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"funder":[{"DOI":"10.13039\/100011022","name":"Vertex Pharmaceuticals","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100011022","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["publications.ersnet.org"],"crossmark-restriction":true},"short-container-title":["ERJ Open Res"],"accepted":{"date-parts":[[2024,5,29]]},"published-print":{"date-parts":[[2024,11]]},"abstract":"<jats:sec><jats:title>Background<\/jats:title><jats:p>People living with cystic fibrosis in Denmark had early, universal access to triple modulator treatment with elexacaftor\/tezacaftor\/ivacaftor. Close monitoring allowed us to assess the impact of treatment on lung function and progression of lung disease in an unselected nationwide cystic fibrosis population from 6\u2005years of age.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Data were analysed using linear mixed-effect models to assess changes in levels and annual rates of change (slopes) in percent predicted (pp) forced expiratory volume in 1\u2005s (FEV<jats:sub>1<\/jats:sub>), forced vital capacity (FVC) and forced expiratory flow at 25\u201375% of FVC (ppFEF<jats:sub>25\u201375%<\/jats:sub>) between the 12\u2005months pre-treatment and treatment periods. Subgroup analyses assessed the impact of elexacaftor\/tezacaftor\/ivacaftor among those with\/without previous modulator treatment, normal\/mild\/moderate\/severe lung disease at treatment initiation, children\/adults and birth cohorts.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>We included 392 people living with cystic fibrosis with a median (interquartile range) 12 (nine to 15) spirometry measurements per person. The mean (95% CI) improvement in ppFEV<jats:sub>1<\/jats:sub>was 13.0 (11.3\u201314.6) 12\u2005months after initiation of elexacaftor\/tezacaftor\/ivacaftor treatment. The annual rate of change improved from \u22121.4 (\u22122.1\u2009\u2013\u2009\u22120.6) ppFEV<jats:sub>1<\/jats:sub>in the pre-treatment year to 2.7 (1.8\u20133.5) ppFEV<jats:sub>1<\/jats:sub>per year during treatment. Similarly, ppFVC increased by 8.0 (7.1\u20138.9) and FEF<jats:sub>25\u2013\u00ad75%<\/jats:sub>by 19.5 (17.0\u201321.9).<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>Using high-resolution data from a nationwide real-world setting, our study documents the impact of elexacaftor\/tezacaftor\/ivacaftor on lung function across subgroups based on age, disease severity and treatment history. These findings point towards a new period of consistent lung function improvement among people living with cystic fibrosis on elexacaftor\/tezacaftor\/ivacaftor.<\/jats:p><\/jats:sec>","DOI":"10.1183\/23120541.00339-2024","type":"journal-article","created":{"date-parts":[[2024,6,20]],"date-time":"2024-06-20T15:25:26Z","timestamp":1718897126000},"page":"00339-2024","update-policy":"https:\/\/doi.org\/10.1183\/ers-crossmark-policy","source":"Crossref","is-referenced-by-count":8,"title":["Lung function improvement on triple modulators: high-resolution, nationwide data from the Danish Cystic Fibrosis Cohort"],"prefix":"10.1183","volume":"10","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-2004-6404","authenticated-orcid":false,"given":"Christian","family":"Leo-Hansen","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Daniel","family":"Faurholt-Jepsen","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tavs","family":"Qvist","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christine","family":"H\u00f8jte","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0622-5262","authenticated-orcid":false,"given":"Bibi U.","family":"Nielsen","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Thomas","family":"Bryrup","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Esben H.","family":"Henriksen","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2233-500X","authenticated-orcid":false,"given":"Terese","family":"Katzenstein","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marianne","family":"Skov","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2398-3575","authenticated-orcid":false,"given":"Inger H.M.","family":"Mathiesen","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Majbritt","family":"Jeppesen","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"S\u00f8ren","family":"Jensen-Fangel","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hanne V.","family":"Olesen","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Frederik Fouirnaies","family":"Buchvald","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kim Gjerum","family":"Nielsen","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Espen","family":"Jimenez-Solem","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christian","family":"Ritz","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tacjana","family":"Pressler","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mette F.","family":"Olsen","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"name":"the TransformCF Study Group","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"81","published-online":{"date-parts":[[2024,6,20]]},"reference":[{"key":"2025011303502692000_10.6.00339-2024.1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jcf.2008.03.009"},{"key":"2025011303502692000_10.6.00339-2024.2","doi-asserted-by":"publisher","DOI":"10.1097\/GIM.0b013e31818e55a2"},{"key":"2025011303502692000_10.6.00339-2024.3","doi-asserted-by":"publisher","DOI":"10.1002\/humu.10041"},{"key":"2025011303502692000_10.6.00339-2024.4","unstructured":"European Cystic Fibrosis Society (ECFS) Patient Registry . 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