Systematic Review of Nasal Endoscopy Scores in Cystic Fibrosis Patients Treated With Cystic Fibrosis Transmembrane...

Cystic fibrosis (CF) is a severe genetic disorder caused by pathogenic variants in the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) gene, leading to multisystem complications including chronic rhinosinusitis and nasal polyposis. Recent advances in CFTR modulator therapies have revolutionized systemic disease control, but their impact on sinonasal disease remains less explored.

A systematic review was conducted following Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines and registered in International Prospective Register of Systematic Reviews (PROSPERO, registration number CRD42025632498). Searches were performed across PubMed/MEDLINE, Cochrane Library, and Embase through March 2025. Studies reporting nasal endoscopic outcomes using validated scoring systems before and after CFTR modulator therapy were included.

Out of 232 identified records, 10 studies met inclusion criteria, representing populations from six countries. Most studies assessed triple therapy (Elexacaftor/Tezacaftor/Ivacaftor). Endoscopic scores, including the Modified Lund-Kennedy scale, showed significant reductions in nasal polyps, mucosal edema, and discharge. Pediatric and adult groups benefited alike, with triple therapy proving more effective than dual or monotherapy. Secondary outcomes included improved pulmonary function, weight gain, and olfactory recovery.

This review demonstrates that CFTR modulators provide significant benefits for sinonasal disease in CF, reinforcing their role as a comprehensive therapeutic approach addressing both upper airway and systemic disease burdens.