Chronic Rhinosinusitis with Nasal Polyposis in People with Cystic Fibrosis

Abstract

Cystic fibrosis (CF) is an autosomal recessive disorder that results in deranged ion transport and affects multiple organ systems, including the upper and lower respiratory tracts. People with CF (PwCF) often develop chronic rhinosinusitis (CRS) with or without nasal polyposis. CRS can significantly decrease quality of life for PwCF and can lead to more frequent pulmonary exacerbations. The management of CRS in PwCF is different from that in individuals without CF. Novel therapies have emerged in the last several years that have drastically altered the progression of both pulmonary and sinonasal disease in people with CF. It is critical for providers who manage CF-related CRS to understand the unique characteristics and challenges that coincide with this disease process. This review article aims to provide readers with an overview of the pathophysiology of CF and to summarize best practice strategies for the management of CF-related CRS.

Keywords

• chronic rhinosinusitis
• nasal polyposis
• cystic fibrosis
• highly effective modulator therapy