Three-drug combo helps manage sinusitis in paediatric cystic fibrosis

Treatment with the fixed-dose combination of elexacaftor, tezacaftor, and ivacaftor (ETI) may reduce the severity of chronic rhinosinusitis in children with cystic fibrosis, as shown in a retrospective study.

The study included 54 children with cystic fibrosis (mean age 11.5 years) treated with ETI. Researchers collected clinical, endoscopic, radiological, functional, and laboratory data at baseline and at the 12-month follow-up.

The primary outcome of chronic rhinosinusitis severity was evaluated using Lund–Kennedy score, Lund–Mackay CT score, and Visual Analogue Scale (VAS). Secondary outcomes included quality of life (CFQ-R), lung function (FEV1), airway microbiology, atopy, and TAS2R38 genotype.

After 12 months of ETI treatment, the patients showed significant improvements in FEV1, CFQ-R, VAS, endoscopic, and radiological CRS scores. None of the patients required sinonasal surgery during follow-up. A significant reduction in Staphylococcus aureus colonization was also observed.

Radiological improvements were markedly smaller in atopic vs nonatopic patients. TAS2R38 genotype was associated with baseline radiological severity, with higher Lund-Mackay scores in AVI/AVI carriers. A genotype-dependent trend in ETI response was observed, with PAV/PAV carriers having smaller radiological improvement.

The findings indicate that atopy and genetic modifiers may influence response to ETI, supporting personalized management of cystic fibrosis-related chronic rhinosinusitis.