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May 2024BACKGROUND
Cystic fibrosis (CF) results from a deficiency or a defect in the CF transmembrane conductance regulator protein (CFTR). Loss of function of the CFTR anion channel causes increased viscosity of secreted mucus and results in significant impairment of the sinonasal mucosa natural mucociliary clearance mechanism. It follows that a high proportion of people with CF (PwCF) are diagnosed with chronic rhinosinusitis (CF-CRS) based on radiographic imaging and/or symptomatology.
Management of CF has changed significantly with the development of CFTR modulator therapy. Ivacaftor was the first commercially available CFTR modulator. Since then, multiple combination medications have been released, including lumacaftor/ivacaftor and tezacaftor/ivacaftor. Most recently, in 2019, elexacaftor/tezacaftor/ivacaftor (ETI) was released as a triple therapy. ETI has been shown to have positive effects on multiple organ systems. Though ETI has continued to show promise in studies pertaining to CF-CRS, guidelines have not strongly endorsed administration of CFTR modulator therapy for the primary indication of CF-CRS. Instead, the most recent Cystic Fibrosis Foundation guidelines only suggest considering CFTR modulator therapy for the isolated indication of CF-CRS. This review outlines recent prospective data on the effect of ETI on CF-CRS and necessitates reconsideration of Cystic Fibrosis Foundation guidelines about this treatment to one that recommends it.
BEST PRACTICE
Best current evidence regarding ETI for patients with PwCF and CF-CRS consists of prospective single-arm cohort studies. ETI is highly effective in improving CT findings, nasal endoscopy findings, and quality of life as measured by various PROMs in both patient groups; however, ETI does not appear to improve olfaction in CF-CRS. Future larger multicenter prospective trials confirming these conclusions are warranted. Given the recent studies outlined above, it is recommended that CFTR modulator therapy not only be considered but used in CF-CRS and PwCF who qualify based on age and genotype.