CORK study in cystic fibrosis: sustained improvements in ultra-low-dose chest CT scores after CFTR modulation with ivacaftor

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dc.check.date2018-10-14
dc.check.infoAccess to this item is restricted for 12 months after publication by request of the publisher.en
dc.contributor.authorRonan, Nicola J.
dc.contributor.authorEinarsson, Gisli G.
dc.contributor.authorTwomey, Maria
dc.contributor.authorMooney, Denver
dc.contributor.authorMullane, David
dc.contributor.authorNiChroinin, Muireann
dc.contributor.authorO'Callaghan, Grace
dc.contributor.authorShanahan, Fergus
dc.contributor.authorMurphy, Desmond M.
dc.contributor.authorO'Connor, Owen J.
dc.contributor.authorShortt, Cathy A.
dc.contributor.authorTunney, Michael M.
dc.contributor.authorEustace, Joseph A.
dc.contributor.authorMaher, Michael M.
dc.contributor.authorElborn, J. Stuart
dc.contributor.authorPlant, Barry J.
dc.contributor.funderSeventh Framework Programmeen
dc.date.accessioned2017-11-16T11:41:00Z
dc.date.available2017-11-16T11:41:00Z
dc.date.issued2017-10-14
dc.date.updated2017-11-13T18:18:27Z
dc.description.abstractBackground: Ivacaftor produces significant clinical benefit in patients with cystic fibrosis (CF) with the G551D mutation. Prevalence of this mutation at the Cork CF Centre is 23%. This study assessed the impact of CFTR modulation on multiple modalities of patient assessment. Methods: Thirty-three patients with the G551D mutation were assessed at baseline and prospectively every 3 months for 1 year after initiation of ivacaftor. Change in ultra-low-dose chest CT scans, blood inflammatory mediators, and the sputum microbiome were assessed. Results: Significant improvements in FEV1, BMI, and sweat chloride levels were observed post-ivacaftor treatment. Improvement in ultra-low-dose CT imaging scores were observed after treatment, with significant mean reductions in total Bhalla score (P < .01), peribronchial thickening (P = .035), and extent of mucous plugging (P < .001). Reductions in circulating inflammatory markers, including interleukin (IL)-1β, IL-6, and IL-8 were demonstrated. There was a 30% reduction in the relative abundance of Pseudomonas species and an increase in the relative abundance of bacteria associated with more stable community structures. Posttreatment community richness increased significantly (P = .03). Conclusions: Early and sustained improvements on ultra-low-dose CT scores suggest it may be a useful method of evaluating treatment response. It paralleled improvement in symptoms, circulating inflammatory markers, and changes in the lung microbiota.en
dc.description.statusPeer revieweden
dc.description.versionAccepted Versionen
dc.format.mimetypeapplication/pdfen
dc.identifier.citationRonan, N. J., Einarsson, G. G., Twomey, M., Mooney, D., Mullane, D., NiChroinin, M., O’Callaghan, G., Shanahan, F., Murphy, D. M., O’Connor, O. J., Shortt, C. A., Tunney, M. M., Eustace, J. A., Maher, M. M., Elborn, J. S. and Plant, B. J. (2017) ‘CORK study in cystic fibrosis: sustained improvements in ultra-low-dose chest CT scores after CFTR modulation with ivacaftor’, Chest, 153(2), pp. 395-403. doi: 10.1016/j.chest.2017.10.005.en
dc.identifier.doi10.1016/j.chest.2017.10.005
dc.identifier.endpage403
dc.identifier.issn0012-3692
dc.identifier.issued2
dc.identifier.journaltitleChesten
dc.identifier.startpage395
dc.identifier.urihttps://hdl.handle.net/10468/5056
dc.identifier.volume153
dc.language.isoenen
dc.publisherElsevier Inc. on behalf of the American College of Chest Physiciansen
dc.relation.projectinfo:eu-repo/grantAgreement/EC/FP7::SP1::HEALTH/603038/EU/Cystic Fibrosis Microbiome-determined Antibiotic Therapy Trial in Exacerbations: Results Stratified./CFMATTERSen
dc.rights© 2017, American College of Chest Physicians. Published by Elsevier Inc. All rights reserved. This manuscript version is made available under the CC BY-NC-ND 4.0 license.en
dc.rights.urihttp://creativecommons.org/licenses/by-nc-nd/4.0/en
dc.subjectCystic fibrosisen
dc.subjectIvacaftoren
dc.subjectG551Den
dc.subjectLow-dose chest CT imagingen
dc.titleCORK study in cystic fibrosis: sustained improvements in ultra-low-dose chest CT scores after CFTR modulation with ivacaftoren
dc.typeArticle (peer-reviewed)en
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