Purpose: 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.
Methods: 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".
Results: 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.
Conclusion: Further studies are warranted to establish consensus for CF-related CRS therapy.
Download full-text PDF |
Source |
|---|---|
| http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8739462 | PMC |
| http://dx.doi.org/10.1007/s00405-021-06875-6 | DOI Listing |
Publication Analysis
Top Keywords
Similar Publications
Increased prevalence of variants and susceptibility to CRS: A real-world study based on Chinese children.
Heliyon
April 2024
Department of Otolaryngology, Head and Neck Surgery, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing, 100045, China.
Background: Chronic Rhinosinusitis is a common disease in children. The main function of CFTR is to maintain the thickness of the mucous layer on the surface of the nasal mucosa. disease-causing variant can cause CFTR protein dysfunction and induce or aggravate chronic infection.
View Article and Find Full Text PDFDual and triple modulator therapy for chronic rhinosinusitis in cystic fibrosis patients.
Rhinology
August 2024
UZ Leuven, Department of Otorhinolaryngology, Head and Neck surgery, Leuven, Belgium; KU Leuven, Department of Neurosciences, Experimental Otorhinolaryngology, Rhinology Research, Leuven, Belgium; KU Leuven, Department of Microbiology, Immunology and Transplantation, Allergy and Clinical Immunology Research Group, Leuven, Belgium.
Background: The introduction of CFTR modulators has changed the landscape in the treatment of cystic fibrosis (CF) and early case series have shown improvements in sinonasal outcomes in this patient population.
Methodology: A real-word data study was performed to evaluate the impact of dual therapy with tezacaftor/ivacaftor (TEZ/IVA) and triple therapy with elexacaftor/tezacaftor/ivacaftor (ELX/TEZ/IVA) on CF-related chronic rhinosinusitis (CRS), by comparing subjective and objective outcome measures at baseline, 12 months after treatment with TEZ/IVA and six months after treatment with ELX/TEZ/IVA.
Results: In total, 43 CF patients, with a mean age of 32 years, were included.
Elexacaftor-Tezacaftor-Ivacaftor Therapy for Chronic Sinus Disease in Cystic Fibrosis.
JAMA Otolaryngol Head Neck Surg
December 2023
Nationwide Children's Hospital, Columbus, Ohio.
Importance: Cystic fibrosis (CF) is a multiorgan genetic disease with progressive upper and lower airway involvement. The effects of CF transmembrane conductance regulator (CFTR) modifier therapies on CF-related upper airway disease, specifically chronic rhinosinusitis (CRS), are not characterized.
Objective: To determine the outcome of elexacaftor-tezacaftor-ivacaftor (ETI) on CRS as measured by changes in sinus computed tomography (CT) metrics and on clinical parameters in individuals with CF.
Upper Airway Disease in Adults with Cystic Fibrosis in the Era of CFTR Modulators.
Laryngoscope
November 2023
Department of Otorhinolaryngology, Head and Neck surgery, UZ Leuven, Leuven, Belgium.
Objectives: Chronic rhinosinusitis (CRS) is prevalent in people with cystic fibrosis (PwCF) and is often refractory to treatments. Uncontrolled CRS might negatively impact the lower airways and the quality of life. The aim of this study is to evaluate the burden of cystic fibrosis (CF)-related CRS in the era of CF transmembrane conductance regulator (CFTR) modulators.
View Article and Find Full Text PDFAlterations in innate immune responses of patients with chronic rhinosinusitis related to cystic fibrosis.
PLoS One
May 2022
Laboratory of Cellular Immunology, Pathology, Faculty of Medicine, University of Brasilia, Brasília, Federal District, Brazil.
The role of phagocytes of children with cystic fibrosis (CF) associated with different phenotypes of chronic rhinosinusitis (CRS) is unclear. The aim of this study was to evaluate the phagocytic capacity of blood neutrophils and monocytes and production of superoxide anion by phagocytes in patients with CF with or without chronic rhinosinusitis and with or without nasal polyps (NP). This cross-sectional study was established in 2015-2017 in a tertiary reference center to the CF treatment, Brasilia, Brazil.
View Article and Find Full Text PDFWant AI Summaries of new PubMed Abstracts delivered to your In-box?
Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!