Reversal of cylindrical bronchial dilatations in a subset of adults with cystic fibrosis treated with elexacaftor-tezacaftor-ivacaftor
Copyright ©The authors 2024. For reproduction rights and permissions contact permissionsersnet.org..
BACKGROUND: This study sought to evaluate the impact of elexacaftor-tezacaftor-ivacaftor (ETI) on lung structural abnormalities in adults with cystic fibrosis (awCF) with a specific focus on the reversal of bronchial dilatations.
METHODS: Chest computed tomography (CT) performed prior to, and ≥12 months after initiation of ETI were visually reviewed for possible reversal of bronchial dilatations. AwCF with and without reversal of bronchial dilatation (the latter served as controls with 3 controls per case) were selected. Visual Brody score, bronchial and arterial diameters, and lung volume were measured on CT.
RESULTS: Reversal of bronchial dilatation was found in 12/235 (5%) awCF treated with ETI. Twelve awCF with and 36 without reversal of bronchial dilatations were further analyzed (male=56%, mean age=31.6±8.5 years, F508del/F508del CFTR =54% and mean %predicted forced expiratory volume in 1 s=58.8%±22.3). The mean±sd Brody score improved overall from 79.4±29.8 to 54.8±32.3 (p<0.001). Reversal of bronchial dilatations was confirmed by a decrease in bronchial lumen diameter in cases from 3.9±0.9 mm to 3.2±1.1 mm (p<0.001), whereas it increased in awCF without reversal of bronchial dilatation (from 3.5±1.1 mm to 3.6±1.2 mm, p=0.002). Reversal of bronchial dilatations occurred in cylindrical (not varicose or saccular) bronchial dilatations. Lung volumes decreased by -6.6±10.7% in awCF with reversal of bronchial dilatation but increased by +2.3±9.6% in controls (p=0.007).
CONCLUSION: Although bronchial dilatations are generally considered irreversible, ETI was associated with reversal, which was limited to the cylindrical bronchial dilatations subtype, and occurred in a small subset of awCF. Initiating ETI earlier in life may reverse early bronchial dilatations.
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CommentIn: Eur Respir J. 2024 Mar 28;63(3):. - PMID 38548272 |
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| Medienart: |
E-Artikel |
| Erscheinungsjahr: |
2024 |
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| Erschienen: |
2024 |
| Enthalten in: |
Zur Gesamtaufnahme - year:2024 |
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| Enthalten in: |
The European respiratory journal - (2024) vom: 08. Feb. |
| Sprache: |
Englisch |
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| Beteiligte Personen: |
Cazier, Paul [VerfasserIn] |
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| Links: |
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| Anmerkungen: |
Date Revised 28.03.2024 published: Print-Electronic CommentIn: Eur Respir J. 2024 Mar 28;63(3):. - PMID 38548272 Citation Status Publisher |
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| doi: |
10.1183/13993003.01794-2023 |
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| PPN (Katalog-ID): |
NLM368209504 |
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| 100 | 1 | |a Cazier, Paul |e verfasserin |4 aut | |
| 245 | 1 | 0 | |a Reversal of cylindrical bronchial dilatations in a subset of adults with cystic fibrosis treated with elexacaftor-tezacaftor-ivacaftor |
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| 500 | |a CommentIn: Eur Respir J. 2024 Mar 28;63(3):. - PMID 38548272 | ||
| 500 | |a Citation Status Publisher | ||
| 520 | |a Copyright ©The authors 2024. For reproduction rights and permissions contact permissionsersnet.org. | ||
| 520 | |a BACKGROUND: This study sought to evaluate the impact of elexacaftor-tezacaftor-ivacaftor (ETI) on lung structural abnormalities in adults with cystic fibrosis (awCF) with a specific focus on the reversal of bronchial dilatations | ||
| 520 | |a METHODS: Chest computed tomography (CT) performed prior to, and ≥12 months after initiation of ETI were visually reviewed for possible reversal of bronchial dilatations. AwCF with and without reversal of bronchial dilatation (the latter served as controls with 3 controls per case) were selected. Visual Brody score, bronchial and arterial diameters, and lung volume were measured on CT | ||
| 520 | |a RESULTS: Reversal of bronchial dilatation was found in 12/235 (5%) awCF treated with ETI. Twelve awCF with and 36 without reversal of bronchial dilatations were further analyzed (male=56%, mean age=31.6±8.5 years, F508del/F508del CFTR =54% and mean %predicted forced expiratory volume in 1 s=58.8%±22.3). The mean±sd Brody score improved overall from 79.4±29.8 to 54.8±32.3 (p<0.001). Reversal of bronchial dilatations was confirmed by a decrease in bronchial lumen diameter in cases from 3.9±0.9 mm to 3.2±1.1 mm (p<0.001), whereas it increased in awCF without reversal of bronchial dilatation (from 3.5±1.1 mm to 3.6±1.2 mm, p=0.002). Reversal of bronchial dilatations occurred in cylindrical (not varicose or saccular) bronchial dilatations. Lung volumes decreased by -6.6±10.7% in awCF with reversal of bronchial dilatation but increased by +2.3±9.6% in controls (p=0.007) | ||
| 520 | |a CONCLUSION: Although bronchial dilatations are generally considered irreversible, ETI was associated with reversal, which was limited to the cylindrical bronchial dilatations subtype, and occurred in a small subset of awCF. Initiating ETI earlier in life may reverse early bronchial dilatations | ||
| 650 | 4 | |a Journal Article | |
| 700 | 1 | |a Chassagnon, Guillaume |e verfasserin |4 aut | |
| 700 | 1 | |a Dhote, Théo |e verfasserin |4 aut | |
| 700 | 1 | |a Da Silva, Jennifer |e verfasserin |4 aut | |
| 700 | 1 | |a Kanaan, Reem |e verfasserin |4 aut | |
| 700 | 1 | |a Honore, Isabelle |e verfasserin |4 aut | |
| 700 | 1 | |a Carlier, Nicolas |e verfasserin |4 aut | |
| 700 | 1 | |a Revel, Marie-Pierre |e verfasserin |4 aut | |
| 700 | 1 | |a Canniff, Emma |e verfasserin |4 aut | |
| 700 | 1 | |a Martin, Clémence |e verfasserin |4 aut | |
| 700 | 1 | |a Burgel, Pierre-Régis |e verfasserin |4 aut | |
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