Effect of elexacaftor/tezacaftor/ivacaftor on airway and systemic inflammation in cystic fibrosis
| العنوان: | Effect of elexacaftor/tezacaftor/ivacaftor on airway and systemic inflammation in cystic fibrosis |
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| المؤلفون: | Michelle Casey, Claudie Gabillard-Lefort, Oisín F McElvaney, Oliver J McElvaney, Tomás Carroll, Ronan C Heeney, Cedric Gunaratnam, Emer P Reeves, Mark P Murphy, Noel G McElvaney |
| المصدر: | Thorax. :thorax-2022 |
| بيانات النشر: | BMJ, 2023. |
| سنة النشر: | 2023 |
| مصطلحات موضوعية: | Pulmonary and Respiratory Medicine |
| الوصف: | Treatment with elexacaftor/tezacaftor/ivacaftor (ETI) has been shown to improve lung function in people with cystic fibrosis (PWCF). However, its biological effects remain incompletely understood. Here we describe alterations in pulmonary and systemic inflammation in PWCF following initiation of ETI. To address this, we collected spontaneously expectorated sputum and matching plasma from PWCF (n=30) immediately prior to ETI therapy, then again at 3 and 12 months. Within 3 months, PWCF demonstrated reduced activity of neutrophil elastase, proteinase three and cathepsin G, and decreased concentrations of interleukin (IL)−1β and IL-8 in sputum, accompanied by decreasedPseudomonasburden and restoration of secretory leukoprotease inhibitor levels. Once treated with ETI, all airway inflammatory markers studied in PWCF had reduced to levels found in matched non-CF bronchiectasis controls. In PWCF with advanced disease, ETI resulted in decreased plasma concentrations of IL-6, C-reactive protein and soluble TNF receptor one as well as normalisation of levels of the acute phase protein, alpha-1 antitrypsin. These data clarify the immunomodulatory effects of ETI and underscore its role as a disease modifier. |
| تدمد: | 1468-3296 0040-6376 |
| الوصول الحر: | https://explore.openaire.eu/search/publication?articleId=doi_________::b442e6df043caaddb162bbb1a6fbd8c0Test https://doi.org/10.1136/thorax-2022-219943Test |
| رقم الانضمام: | edsair.doi...........b442e6df043caaddb162bbb1a6fbd8c0 |
| قاعدة البيانات: | OpenAIRE |
| تدمد: | 14683296 00406376 |
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