Sinus Disease Grading on Computed Tomography Before and After Modulating Therapy in Adult Patients with Cystic Fibrosis

Abstract

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 sinus disease on Computed Tomography (CT) in cystic fibrosis (CF) patients with G551D mutation. The aim of this study was to evaluate the effect of CFTR modulator therapy in sinus disease using CT scores in a wider CF patient population. Materials and Methods: Forty-eight adult patients with CF underwent at least one CT sinus examination before CFTR modulator therapy (ivacaftor, lumacaftor, ivacaftor/lumacaftor or elexcaftor/tezacaftor/ivacaftor) and one CT sinus examination one year after CFTR modulator therapy initiation. Two radiologists assessed the images in consensus. The Lund-Mackay score (LM score) and the Sheikh-Lind CT sinus disease severity scoring system (SL score) were used. The 22-item SinoNasal Outcome Test (SNOT-22) questionnaire was evaluated before CFTR modulator therapy and one year after CFTR modulator therapy initiation. Results: 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). Conclusion: Evolution of imaging findings on CT during follow-up closely correlate with improved SNOT-22 score one year after CFTR modulator therapy initiation, indicating that CT may be a useful adjunct during follow-up of CF patients under this treatment as an objective measure of sinonasal disease improvement.

Triple combination CFTR modulator therapy reduces the need for endoscopic sinus surgery in adult patients with cystic fibrosis

“…The recently approved triple combination therapy is a potential treatment for many patients who had previously been excluded from CFTR modulation therapy, and previously published articles showed that ETI improves both symptoms and radiological findings of sinonasal disease. [1][2][3] ESS is a minimally invasive procedure performed through the nostrils that consists of enlarging sinus ostia, removing causes of sinus blockage, such as nasal polyps, and restoring adequate aeration of sinuses.…”

“…Sinonasal involvement causes frequent symptoms and a significant quality of life worsening in CF patients. The recently approved triple combination therapy is a potential treatment for many patients who had previously been excluded from CFTR modulation therapy, and previously published articles showed that ETI improves both symptoms and radiological findings of sinonasal disease 1–3 …”

“…Therefore, this treatment provides potential therapy to many patients who had previously been excluded from CFTR modulation therapy. Previous studies showed the clinical effectiveness of ETI in chronic rhinosinusitis of CF patients 1–3 …”

“…Previous studies showed the clinical effectiveness of ETI in chronic rhinosinusitis of CF patients. [1][2][3] The aim of this study was to evaluate the association between triple combination therapy and the number of endoscopic sinus surgery (ESS) procedures performed in CF patients with chronic rhinosinusitis.…”

Dual and triple modulator therapy for chronic rhinosinusitis in cystic fibrosis patients

“…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.…”