{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,11,10]],"date-time":"2026-11-10T17:10:44Z","timestamp":1794330644167,"version":"4.9.0"},"reference-count":48,"publisher":"Oxford University Press (OUP)","issue":"11","license":[{"start":{"date-parts":[[2023,12,1]],"date-time":"2023-12-01T00:00:00Z","timestamp":1701388800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/academic.oup.com\/pages\/standard-publication-reuse-rights"}],"funder":[{"name":"German Federal Ministry of Education and Research","award":["82DZL00401"],"award-info":[{"award-number":["82DZL00401"]}]},{"name":"German Federal Ministry of Education and Research","award":["82DZL004A1"],"award-info":[{"award-number":["82DZL004A1"]}]},{"name":"German Federal Ministry of Education and Research","award":["82DZL009B1"],"award-info":[{"award-number":["82DZL009B1"]}]},{"DOI":"10.13039\/501100001659","name":"German Research Foundation","doi-asserted-by":"publisher","award":["STA 1685\/1-1"],"award-info":[{"award-number":["STA 1685\/1-1"]}],"id":[{"id":"10.13039\/501100001659","id-type":"DOI","asserted-by":"publisher"}]},{"name":"Mukoviszidose e.V.","award":["S02\/09"],"award-info":[{"award-number":["S02\/09"]}]},{"name":"Mukoviszidose e.V.","award":["C-H-P 1504"],"award-info":[{"award-number":["C-H-P 1504"]}]},{"name":"German Federal Ministry of Education and Research","award":["82DZL00401"],"award-info":[{"award-number":["82DZL00401"]}]},{"name":"German Federal Ministry of Education and Research","award":["82DZL004A1"],"award-info":[{"award-number":["82DZL004A1"]}]},{"name":"German Federal Ministry of Education and Research","award":["82DZL009B1"],"award-info":[{"award-number":["82DZL009B1"]}]},{"DOI":"10.13039\/501100001659","name":"German Research Foundation","doi-asserted-by":"crossref","award":["STA 1685\/1-1"],"award-info":[{"award-number":["STA 1685\/1-1"]}],"id":[{"id":"10.13039\/501100001659","id-type":"DOI","asserted-by":"crossref"}]},{"name":"Mukoviszidose e.V.","award":["S02\/09"],"award-info":[{"award-number":["S02\/09"]}]},{"name":"Mukoviszidose e.V.","award":["C-H-P 1504"],"award-info":[{"award-number":["C-H-P 1504"]}]}],"content-domain":{"domain":["www.atsjournals.org"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2023,12,1]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Rationale<\/jats:title>\n                    <jats:p>Magnetic resonance imaging (MRI) detects improvements in mucus plugging and bronchial wall thickening, but not in lung perfusion in patients with cystic fibrosis (CF) treated with elexacaftor\/tezacaftor\/ivacaftor (ETI).<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Objectives<\/jats:title>\n                    <jats:p>To determine whether bronchial artery dilatation (BAD), a key feature of advanced lung disease, indicates irreversibility of perfusion abnormalities and whether BAD could be reversed in CF patients treated with ETI.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>A total of 59 adults with CF underwent longitudinal chest MRI, including magnetic resonance angiography twice, comprising 35 patients with CF (mean age, 31\u2009\u00b1\u20097\u2009yr) before (MRI1) and after (MRI2) at least 1\u2009month (mean duration, 8\u2009\u00b1\u20094\u2009mo) on ETI therapy and 24 control patients with CF (mean age, 31\u2009\u00b1\u20097\u2009yr) without ETI. MRI was assessed using the validated chest MRI score, and the presence and total lumen area of BAD were assessed with commercial software.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>The MRI global score was stable in the control group from MRI1 to MRI2 (mean difference, 1.1 [\u22120.3, 2.4]; P\u2009=\u20090.054), but it was reduced in the ETI group (\u221210.1 [\u22120.3, 2.4]; P\u2009&amp;lt;\u20090.001). In the control and ETI groups, BAD was present in almost all patients at baseline (95% and 94%, respectively), which did not change at MRI2. The BAD total lumen area did not change in the control group from MRI1 to MRI2 (1.0 mm2 [\u22120.2, 2.2]; P\u2009=\u20090.099) but decreased in the ETI group (\u22127.0 mm2 [\u22128.9, \u22125.0]; P\u2009&amp;lt;\u20090.001). This decrease correlated with improvements in the MRI global score (r\u2009=\u20090.540; P\u2009&amp;lt;\u20090.001).<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>Our data show that BAD may be partially reversible under ETI therapy in adult patients with CF who have established disease.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1513\/annalsats.202302-168oc","type":"journal-article","created":{"date-parts":[[2023,8,14]],"date-time":"2023-08-14T16:06:57Z","timestamp":1692029217000},"page":"1595-1604","update-policy":"https:\/\/doi.org\/10.1164\/crossmarkpolicies","source":"Crossref","is-referenced-by-count":9,"title":["Elexacaftor\/Tezacaftor\/Ivacaftor Improves Bronchial Artery Dilatation Detected by Magnetic Resonance Imaging in Patients with Cystic Fibrosis"],"prefix":"10.1093","volume":"20","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-2280-1269","authenticated-orcid":false,"given":"Lena","family":"Wucherpfennig","sequence":"first","affiliation":[{"name":"Department of Diagnostic and Interventional Radiology"},{"name":"Department of Diagnostic and Interventional Radiology with Nuclear Medicine, and"},{"name":"Translational Lung Research Center Heidelberg, German Center for Lung Research, Heidelberg, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Simon M. F.","family":"Triphan","sequence":"additional","affiliation":[{"name":"Department of Diagnostic and Interventional Radiology"},{"name":"Department of Diagnostic and Interventional Radiology with Nuclear Medicine, and"},{"name":"Translational Lung Research Center Heidelberg, German Center for Lung Research, Heidelberg, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sabine","family":"Wege","sequence":"additional","affiliation":[{"name":"Department of Pulmonology and Respiratory Medicine, Cystic Fibrosis Center, Thoracic Clinic, University Hospital Heidelberg, Heidelberg, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hans-Ulrich","family":"Kauczor","sequence":"additional","affiliation":[{"name":"Department of Diagnostic and Interventional Radiology"},{"name":"Department of Diagnostic and Interventional Radiology with Nuclear Medicine, and"},{"name":"Translational Lung Research Center Heidelberg, German Center for Lung Research, Heidelberg, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Claus P.","family":"Heussel","sequence":"additional","affiliation":[{"name":"Department of Diagnostic and Interventional Radiology"},{"name":"Department of Diagnostic and Interventional Radiology with Nuclear Medicine, and"},{"name":"Translational Lung Research Center Heidelberg, German Center for Lung Research, Heidelberg, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Olaf","family":"Sommerburg","sequence":"additional","affiliation":[{"name":"Translational Lung Research Center Heidelberg, German Center for Lung Research, Heidelberg, Germany"},{"name":"Department of Translational Pulmonology and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mirjam","family":"Stahl","sequence":"additional","affiliation":[{"name":"Translational Lung Research Center Heidelberg, German Center for Lung Research, Heidelberg, Germany"},{"name":"Department of Translational Pulmonology and"},{"name":"Division of Pediatric Pulmonology and Allergy and Cystic Fibrosis Center, University of Heidelberg, Heidelberg, Germany"},{"name":"Department of Pediatric Respiratory Medicine, Immunology and Critical Care Medicine, Charit\u00e9 \u2013 University Medicine Berlin, corporate member of Freie Universit\u00e4t Berlin and Humboldt-Universit\u00e4t zu Berlin, Berlin, Germany"},{"name":"German Center for Lung Research, Berlin, Germany; and"},{"name":"Berlin Institute of Health at Charit\u00e9 \u2013 University Medicine Berlin, Berlin, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4057-2199","authenticated-orcid":false,"given":"Marcus A.","family":"Mall","sequence":"additional","affiliation":[{"name":"Translational Lung Research Center Heidelberg, German Center for Lung Research, Heidelberg, Germany"},{"name":"Department of Translational Pulmonology and"},{"name":"Division of Pediatric Pulmonology and Allergy and Cystic Fibrosis Center, University of Heidelberg, Heidelberg, Germany"},{"name":"Department of Pediatric Respiratory Medicine, Immunology and Critical Care Medicine, Charit\u00e9 \u2013 University Medicine Berlin, corporate member of Freie Universit\u00e4t Berlin and Humboldt-Universit\u00e4t zu Berlin, Berlin, Germany"},{"name":"German Center for Lung Research, Berlin, Germany; and"},{"name":"Berlin Institute of Health at Charit\u00e9 \u2013 University Medicine Berlin, Berlin, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Monika","family":"Eichinger","sequence":"additional","affiliation":[{"name":"Department of Diagnostic and Interventional Radiology"},{"name":"Department of Diagnostic and Interventional Radiology with Nuclear Medicine, and"},{"name":"Translational Lung Research Center Heidelberg, German Center for Lung Research, Heidelberg, 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