Management of chronic rhinosinusitis in CF

Abstract

Routine CF management often does not include upper airway (UAW) assessment although CFTR defects equally affect the sinonasal mucosa. Up to 50% of CF patients have chronic rhinosinusitis (CRS) and/or nasal polyps, and almost 100% reveal UAW abnormalities on CT scan. CRS impairs quality of life. UAW dysfunction in filtering, humidifying, and warming inspired air affects lower airways and the UAW is a potential site of first colonization and a reservoir for opportunistic bacteria. Therefore, UAW pathology substantially affects overall health in CF. Standard treatments are scarce and mostly lack evidence. Nasal douche can remove mucus and crusts. Recently, delivery of dornase alfa using a vibrating aerosol has shown potential as treatment for CF-related CRS. Surgery is indicated when conservative approaches fail but postoperative relapse is frequent. In summary, upper airway involvement in CF is undertreated and requires prospective investigation and an interdisciplinary consensus on diagnosis and therapy.