Chronic rhinosinusitis in cystic fibrosis: a review of therapeutic options.

Joanna Krajewska, Krzysztof Zub, Adam Słowikowski, Tomasz Zatoński

European archives of oto-rhino-laryngology : official journal of the European Federation of Oto-Rhino-Laryngological Societies (EUFOS) : affiliated with the German Society for Oto-Rhino-Laryngology - Head and Neck Surgery 2022 Jan

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Chronic rhinosinusitis (CRS) is observed in almost 100% of patients with cystic fibrosis (CF). CF-related CRS treatment is extremely challenging because of the underlying genetic defect leading to its development. CRS in CF is often refractory to standard therapy, while recurrences after surgical treatment are inevitable in the majority of patients. This study provides a precise review of the current knowledge regarding possible therapeutic options for CF-related CRS. The Medline and Web of Science databases were searched without a time limit using the terms "cystic fibrosis" in conjunction with "otorhinolaryngological manifestation", "rhinology" and "sinusitis". Precise guidelines for CF-induced CRS therapy are lacking due to the lack of large cohort randomized controlled trials. None of the existing therapeutic agents has already been recommended for CRS in CF. Therapy targeting the underlying genetic defect, intranasal dornase alfa administration, and topical delivery of colistin and tobramycin showed promising results in CF-related CRS therapy. Besides the potential effectiveness of nasal steroids, strong recommendations for their usage in CF have not been provided yet. Systemic corticosteroid usage is controversial due to its potential negative influence on pulmonary disease. Ibuprofen revealed some positive effects on CF-related CRS in molecular and small cohort studies. Intranasal irrigation with saline solutions could relieve sinonasal symptoms. Nasal decongestants are not recommended. Endoscopic sinus surgery is the first-line surgical option for refractory CRS. Extensive surgical approaches should be considered as they could improve long-term outcomes in CRS. Further studies are warranted to establish consensus for CF-related CRS therapy. © 2021. The Author(s).

Citation

Joanna Krajewska, Krzysztof Zub, Adam Słowikowski, Tomasz Zatoński. Chronic rhinosinusitis in cystic fibrosis: a review of therapeutic options. European archives of oto-rhino-laryngology : official journal of the European Federation of Oto-Rhino-Laryngological Societies (EUFOS) : affiliated with the German Society for Oto-Rhino-Laryngology - Head and Neck Surgery. 2022 Jan;279(1):1-24