{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,25]],"date-time":"2026-08-25T04:09:20Z","timestamp":1787630960530,"version":"build-2736575974"},"reference-count":15,"publisher":"Wiley","issue":"7","license":[{"start":{"date-parts":[[2023,6,12]],"date-time":"2023-06-12T00:00:00Z","timestamp":1686528000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Respirology Case Reports"],"published-print":{"date-parts":[[2023,7]]},"abstract":"Abstract<\/jats:title>\n \n Bronchiectasis is often considered progressive and irreversible, so cases of regression or reversal are an important step in understanding the underlying pathophysiological mechanisms. Cystic fibrosis, (CF) caused by pathogenic variants in the\n cystic fibrosis transmembrane conductance regulator<\/jats:italic>\n (\n CFTR<\/jats:italic>\n ) gene has been a success story in personalized medicine. The recent development of\n CFTR<\/jats:italic>\n modulator therapies has revolutionized care. Dramatic improvements in lung function, sputum production, daytime functioning, and quality of life are seen within weeks. However, the effect of long\u2010term exposure to elexacaftor + tezacaftor + ivacaftor (ETI) on the structural abnormalities is at present unknown. This case series outlines three adults with CF who have demonstrated progressive improvement in the cylindrical, varicose and importantly cystic changes of bronchiectasis with prolonged ETI treatment. This raises the exciting question of reversibility of bronchiectasis as well as the mechanisms involved in the maintenance and progression of bronchiectasis as it relates to CF.\n <\/jats:p>","DOI":"10.1002\/rcr2.1172","type":"journal-article","created":{"date-parts":[[2023,6,12]],"date-time":"2023-06-12T23:42:06Z","timestamp":1686613326000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":20,"title":["Cystic fibrosis modulator therapy can reverse cystic bronchiectasis"],"prefix":"10.1002","volume":"11","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-3361-4941","authenticated-orcid":false,"given":"Peter G.","family":"Middleton","sequence":"first","affiliation":[{"name":"CF Service, Department Respiratory & Sleep Medicine Westmead Hospital Sydney New South Wales Australia"},{"name":"Westmead Clinical School University of Sydney Sydney New South Wales Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1705-4780","authenticated-orcid":false,"given":"Nicholas J.","family":"Simmonds","sequence":"additional","affiliation":[{"name":"Adult Cystic Fibrosis Centre Royal Brompton Hospital London UK"},{"name":"Respiratory Medicine Imperial College London, Royal Brompton Hospital London UK"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2023,6,12]]},"reference":[{"key":"e_1_2_9_2_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMra012519"},{"key":"e_1_2_9_3_1","first-page":"6","article-title":"Inflammation: a two\u2010edged sword\u2014the model of bronchiectasis","volume":"147","author":"Cole PJ","year":"1986","journal-title":"Eur J Respir Dis Suppl"},{"key":"e_1_2_9_4_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMra2202819"},{"key":"e_1_2_9_5_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(18)31767-7"},{"key":"e_1_2_9_6_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1908639"},{"key":"e_1_2_9_7_1","doi-asserted-by":"publisher","DOI":"10.1080\/17476348.2021.1855980"},{"key":"e_1_2_9_8_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1301725"},{"key":"e_1_2_9_9_1","doi-asserted-by":"publisher","DOI":"10.1378\/chest.07-1529"},{"key":"e_1_2_9_10_1","doi-asserted-by":"publisher","DOI":"10.1164\/ajrccm.186.3.292"},{"key":"e_1_2_9_11_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jcf.2014.06.011"},{"key":"e_1_2_9_12_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jcf.2022.03.011"},{"key":"e_1_2_9_13_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ejrad.2022.110421"},{"key":"e_1_2_9_14_1","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.200508-1330OC"},{"key":"e_1_2_9_15_1","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.201304-0733OC"},{"key":"e_1_2_9_16_1","doi-asserted-by":"publisher","DOI":"10.1183\/13993003.01581-2021"}],"container-title":["Respirology Case Reports"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/rcr2.1172","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,8,17]],"date-time":"2023-08-17T15:09:14Z","timestamp":1692284954000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/rcr2.1172"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2023,6,12]]},"references-count":15,"journal-issue":{"issue":"7","published-print":{"date-parts":[[2023,7]]}},"alternative-id":["10.1002\/rcr2.1172"],"URL":"https:\/\/doi.org\/10.1002\/rcr2.1172","archive":["Portico"],"relation":{},"ISSN":["2051-3380","2051-3380"],"issn-type":[{"value":"2051-3380","type":"print"},{"value":"2051-3380","type":"electronic"}],"subject":[],"published":{"date-parts":[[2023,6,12]]},"assertion":[{"value":"2023-03-13","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2023-05-23","order":1,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2023-06-12","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}],"article-number":"e01172"}}