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Axial sinus computed tomography images from a cystic fibrosis patient with chronic rhinosinusitis before (a) and after (b) one year of modulating therapy.
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Objectives:
Cystic fibrosis transmembrane conductance regulator (CFTR) modulator therapy effects on respiratory function, pulmonary exacerbations and quality of life have been well documented. However, CFTR modulator therapy effects on sinus disease have not been so well reported. A previous study reported that ivacaftor improves appearance of sin...
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Context 1
... LM, SL and SNOT-22 scores one year after CFTR modulator therapy were respectively 8.0 ± 2.7, 6.5 ± 1.8 and 16.2 ± 6.5. CT sinus examination after CFTR modulator therapy showed statistically significant lower mean LM, SL and SNOT-22 scores than CT sinus examination before CFTR modulator therapy (p < 0.001) (Figures 1, 2 and 3). ...
Citations
... Shortly after the introduction of IVA, the first reports indicated not only amelioration in pulmonary health but also notable improvements in cystic fibrosis-related secondary chronic rhinosinusitis (CRS), especially in patients with at least one G551D gating mutation (11) . Studies on ELX/TEZ/IVA, both prospective and retrospective, revealed substantial reductions in sinonasal complaints and radiographical disease severity (12)(13)(14)(15)(16)(17)(18)(19) . To date, no clinical data are available on the impact of TEZ/IVA dual therapy on CF-related secondary CRS or the effect of modulator therapy on nasal endoscopy or bacterial upper airway colonization. ...
... In Corrected Proof studies, showing that ELX/TEZ/IVA significantly reduces SNOT-22 (12)(13)(14)(15)(16)(18)(19) and Lund-Mackay scores (12-13, 17, 19) . It should be noted that the magnitude of reduction in SNOT-22 score of -6.8 points is rather limited in comparison to current literature, with a reported mean change in SNOT-22 score ranging from -10.5 to -15 (12)(13)(14)(15)(16)(18)(19), and might not be clinically meaningful. ...
... In Corrected Proof studies, showing that ELX/TEZ/IVA significantly reduces SNOT-22 (12)(13)(14)(15)(16)(18)(19) and Lund-Mackay scores (12-13, 17, 19) . It should be noted that the magnitude of reduction in SNOT-22 score of -6.8 points is rather limited in comparison to current literature, with a reported mean change in SNOT-22 score ranging from -10.5 to -15 (12)(13)(14)(15)(16)(18)(19), and might not be clinically meaningful. This might be due to low SNOT-22 scores at baseline (24.4 ± 15.2 points). ...
Background: The introduction of CFTR modulators has changed the landscape in the treatment of cystic fibrosis (CF) and early case series have shown improvements in sinonasal outcomes in this patient population. Methodology: A real-word data study was performed to evaluate the impact of dual therapy with tezacaftor/ivacaftor (TEZ/IVA) and triple therapy with elexacaftor/tezacaftor/ivacaftor (ELX/TEZ/IVA) on CF-related chronic rhinosinusitis (CRS), by comparing subjective and objective outcome measures at baseline, 12 months after treatment with TEZ/IVA and six months after treatment with ELX/TEZ/IVA. Results: In total, 43 CF patients, with a mean age of 32 years, were included. After triple therapy, significant improvements in overall visual analogue scale, SNOT-22, Lund Kennedy, nasal polyps, and Lund-Mackay scores were observed, whereas no beneficial effect could be seen in patients treated with dual therapy. Bacterial upper airway colonization did not differ pre- and postmodulator therapy in the present study. The number of responders to dual and triple therapy is 23.8% and 63.2% of the patients, respectively. Conclusions: Triple therapy with ELX/TEZ/IVA is superior to dual therapy with TEZ/IVA in the treatment of CF-CRS, as significantly reduced sinonasal complaints, nasal endoscopy and CT scores were observed after triple therapy, whereas this was not the case for dual therapy.
... В 2022 г. в одном из изданий, посвященных лучевой диагностике, появляется описание динамики КТ-картины 148 взрослых пациентов, получавших таргетную терапию МВ (без уточнения препаратов). Продемонстрирована высокая степень корреляции качества жизни, оцененной с помощью опросника SNOT-22, и рентгенологической характеристики ОНП [18]. ...
Introduction. Chronic rhinosinusitis (CRS) with underlying cystic fibrosis (CF) is one of the most challenging inflammatory diseases of the paranasal sinuses (PNS). The treatment of CF in general and CF-associated CRS in particular keeps developing exponentially: special drug delivery systems have been invented, drugs purpose-developed for the treatment of patients with CF (dornase alfa) are used, and radicality of surgical intervention is increasing. In recent years, CF innovative targeted therapy has been introduced. It aims to improve the course of the bronchopulmonary infectious and inflammatory process, nutritional status and pancreatic function, but its efficacy in relation to the PNS condition is still insufficiently studied.
Aim. To evaluate the efficacy of ivacaftor/elexacaftor/tezacaftor therapy for CF sinonasal manifestations in children and to determine possible parallels of the CRS course vs other characteristics of the CF course.
Mat erials and methods. A total of 15 children with CF receiving triple targeted therapy were included in the first series of observations. Computed tomography (CT) scans were assessed using the Lund-Mackay scores (LMS) before and 7.6 ± 3.0 months after starting the therapy. In addition, the changes in sweat chloride levels and pulmonary function test results were taken into account. The control group included 11 patients with comparable initial radiographic data who did not receive such therapy.
Results and discussion. The treatment group showed a significant improvement in the PNS condition in the form of a decrease in the LMS scores from 15.6 ± 4.5 to 2.1 ± 3.0 (p = 0.001) with inhomogeneous changes vs different PNS groups. A certain parallelism between changes in the CT view and the level of sweat chlorides and pulmonary function test results was reported. In the control group, a nonsignificant deterioration in the course of CRS was observed (from 12.3 ± 6.3 to 14.6 ± 4.9 scores according to LMS; p = 0.108).
Conclusions. The study demonstrated high efficacy of CF triple targeted therapy with ivacaftor/elexacaftor/tezacaftor in relation to the severe CRS with a rapid onset of effect in the form of PNS pneumatization restoration according to CT findings.
Chronic rhinosinusitis is a common manifestation of CF that is associated with impaired quality of life and can be difficult to treat. CFTR modulator therapy has resulted in significant improvements in lower respiratory and nutritional outcomes for people with CF however their impact on chronic rhinosinusitis has received less attention. We review the literature in relation to chronic rhinosinusitis in CF and examine the impact of CFTR modulator therapy on symptoms, imaging, endoscopic appearances, and olfactory outcomes in the treatment of chronic rhinosinusitis. While an overall improvement in symptoms, imaging and endoscopic appearances is seen in response to treatment, limited impact is documented on olfaction. Outcome measures employed were heterogenous, limiting comparison of findings. There is a need for well powered prospective real-world studies with standardised outcome measures.
