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J Bras Pneumol. 2015 Jan-Feb;41(1):65-76. doi: 10.1590/S1806-37132015000100009.

Chronic rhinosinusitis and nasal polyposis in cystic fibrosis: update on diagnosis and treatment.

Author information

1
Federal University of Rio Grande do Sul, School of Medicine, Porto Alegre, Brazil. Graduate Program in Pulmonology, Federal University of Rio Grande do Sul School of Medicine, Porto Alegre, Brazil.
2
Federal University of Rio Grande do Sul, School of Medicine, Department of Ophthalmology and Otolaryngology, Porto Alegre, Brazil. Department of Ophthalmology and Otolaryngology, Federal University of Rio Grande do Sul School of Medicine, Porto Alegre, Brazil.
3
Federal University of Rio Grande do Sul, School of Medicine, Department of Otolaryngology and Head & Neck Surgery, Porto Alegre, Brazil. Department of Otolaryngology and Head & Neck Surgery, Federal University of Rio Grande do Sul School of Medicine Hospital de Clínicas, Porto Alegre, Brazil.

Abstract

in English, Portuguese

Although cystic fibrosis (CF) is an irreversible genetic disease, advances in treatment have increased the life expectancy of CF patients. Upper airway involvement, which is mainly due to pathological changes in the paranasal sinuses, is prevalent in CF patients, although many are only mildly symptomatic (with few symptoms). The objective of this literature review was to discuss the pathophysiology and current therapeutic management of chronic rhinosinusitis (CRS) in CF patients. The review was based on current evidence, which was classified in accordance with the Oxford Centre for Evidence-Based Medicine criteria. When symptomatic, CRS with nasal polyps can affect quality of life and can lead to pulmonary exacerbations, given that the paranasal sinuses can be colonized with pathogenic bacteria, especially Pseudomonas aeruginosa. Infection with P. aeruginosa plays a crucial role in morbidity and mortality after lung transplantation in CF patients. Although clinical treatment of the upper airways is recommended as initial management, this recommendation is often extrapolated from studies of CRS in the general population. When sinonasal disease is refractory to noninvasive therapy, surgery is indicated. Further studies are needed in order to gain a better understanding of upper airway involvement and improve the management of CRS in CF patients, with the objective of preserving lung function and avoiding unnecessary invasive procedures.

KEYWORDS:

Cystic fibrosis; Nasal polyps; Nose diseases; Paranasal sinuses; Sinusitis

PMID:
25750676
PMCID:
PMC4350827
DOI:
10.1590/S1806-37132015000100009
[Indexed for MEDLINE]
Free PMC Article

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