Upper Airway Disease in Adults with Cystic Fibrosis in the Era of CFTR Modulators

• Published in: The Laryngoscope 2023-11, Vol.133 (11), p.2898-2909
• Main Authors: Uyttebroek, Saartje, Dupont, Lieven, Jorissen, Mark, Van Gerven, Laura
• Format: Article
• Language: English
• Subjects: CFTR modulator; chronic rhinosinusitis; Cystic fibrosis; Endoscopy; nasal polyps; upper airway disease
• ISSN: 0023-852X; 1531-4995
• DOI: 10.1002/lary.30642

Objectives Chronic rhinosinusitis (CRS) is prevalent in people with cystic fibrosis (PwCF) and is often refractory to treatments. Uncontrolled CRS might negatively impact the lower airways and the quality of life. The aim of this study is to evaluate the burden of cystic fibrosis (CF)‐related CRS in the era of CF transmembrane conductance regulator (CFTR) modulators. Methods Adult PwCF were asked to fill in a questionnaire on sinonasal complaints, they underwent a nasal endoscopy, bacteriological sampling, and a CT scan. Afterwards, these outcome measures were compared between patients treated with and without modulators. Results In the 122 included patients, CRS was present in 83%. CFTR modulators were prescribed in 48% of the patients, with a median of 10 months since the start of the treatment. Subjectively, the median SNOT‐22 score was 16/110. Objectively, a median Lund‐Kennedy score of 6/12 and modified Lund‐Mackay score of 10/24 were observed. No correlation could be found between SNOT‐22 score and other outcome measures including endoscopy and radiology. Altogether, 21% of the patients had controlled disease. When comparing patients treated with and without modulators, significantly lower CT scores (p = 0.0018) and less bacterial colonization (p = 0.0082) were observed in patients receiving modulators. Conclusion CF‐CRS is highly prevalent in our cohort and only the minority of PwCF has a well‐controlled disease. A multidisciplinary ENT‐pneumology clinic would be beneficial, as there is a high discrepancy between patient‐reported symptoms and the extent of the disease. CFTR modulators are promising, as lower CT scores and less bacterial colonization were observed in the modulator group. Level of Evidence Level 3 Laryngoscope, 133:2898–2909, 2023 The introduction of CFTR modulators has changed the landscape in cystic fibrosis. The aim of this study was to evaluate the burden of sinus disease in adult patients with cystic fibrosis in the era of CFTR modulators, including the impact on the quality of life and objective extent of the disease. Summarized, the diagnosis of CF‐related chronic rhinosinusitis was made in 83% of the patients and despite the presence of sinonasal complaints in almost half of them, patient‐reported scores were usually low and did not correlate with objective disease severity. CFTR modulators are promising as significantly lower CT scores and fewer bacterial colonization were observed in the subgroup of patients treated with mod