Sinonasal manifestations of cystic fibrosis: A correlation between genotype and phenotype?

Background: Patients with Cystic Fibrosis are prone to develop sinonasal disease. Studies in genotype phenotype correlations for sinonasal disease are scarce and inconclusive. Methods: In this observational study several aspects of sinonasal disease were investigated in 104 adult patients with CF. In each patient a disease specific quality of life questionnaire (RSOM-31), nasal endoscopy and a CT scan of the paranasal sinuses were performed. Patients were divided into two groups, clash I-III mutations and class IV-V mutations, based on their CFTR mutations. Results: The prevalence of rhinosinusitis in adult patients with CF was 63% and the prevalence of nasal polyps 25%. Patients with class I-III mutations had significantly smaller frontal sinuses, sphenoid sinuses, more opacification in the sinonasal area and more often osteitis/neoosteogenesis of die maxillary sinus wall compared to patients with class IV and V mutations. Conclusion: These data suggest more severe sinonasal disease in patients with class I-III mutations compared to patients with class IV-V mutations. (C) 2013 European Cystic Fibrosis Society. Published by Elsevier B.V. All rights reserved.