Pathogenesis and Management of Nasal Polyposis in Cystic Fibrosis
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Beginning in preschool age, during their lives, up to 50% of cystic fibrosis (CF) patients experience obstructing nasal polyposis (NP), which is rare in non-CF children. Pathogenetic factors of NP in general and especially in CF are still obscure. However, defective epithelial ion transport from mucosal glands plays a central role in CF, and viscous secretions impair mucociliary clearance, promoting chronic pathogen colonization and neutrophil-dominated chronic inflammation. Presently, CF-NP is not curable but can be clinically stabilized, though the large variety of proposed treatment modalities indicates a lack of standardization and of evidence of treatment efficacy. When conservative measures are exhausted, surgical intervention combining individually adapted endoscopic sinus surgery and supportive conservative treatment is performed. Topical steroids, approved as the gold standard for non-CF NP, may be beneficial, but they are discussed to be less effective in neutrophilic inflammation, and CF-specific antimicrobial and mucolytic therapy, as is true of all treatment modalities, urgently requires evaluation by controlled clinical trials within interdisciplinary networks.
KeywordsNasal polyposis Cystic fibrosis Upper airways Rhinosinusitis Nose Ostiomeatal complex Inflammation Mucociliary clearance Pathogen colonization Pseudomonas aeruginosa Staphylococcus aureus Topical therapy Steroids Mucolytics Antibiotics Macrolides Sinonasal inhalation Nasal lavage Rhinoscopy Sinonasal surgery FESS Neutrophil Interleukins IL-8 Quality of life
The authors thank Franziska Doht and Dr. Ruth Michl for their support in preparation of the manuscript.
Dr. Mainz has served on advisory boards for Grunenthal, Gilead, and Pharmaxis; has had travel expenses covered by Novartis, Pari, Roche, Infectopharm, and MSD; and has conducted clinical trials on behalf of Luxemburgian and Germany CF Foundations and Pari Germany, Roche, Infectopharm, and Forest.
Dr. Koitschev reported no potential conflicts of interest relevant to this article.
Papers of particular interest, published recently, have been highlighted as: • Of importance •• Of major importance
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