New insights into the pathogenesis of cystic fibrosis sinusitis

EH Chang - International forum of allergy & rhinology, 2014 - Wiley Online Library
EH Chang
International forum of allergy & rhinology, 2014Wiley Online Library
Background People with cystic fibrosis (CF) sinus disease have developmental sinus
abnormalities with airway bacterial infection, inflammation, impaired mucociliary clearance
and thick obstructive mucus. The pathophysiology of airway disease in CF is not completely
understood, and current treatments in CF sinus disease ameliorate symptoms but do not
provide a cure. Methods This manuscript reviews the history of CF, its manifestations in
sinus disease, and the potential impact and relationship of CF on the upper and lower …
Background
People with cystic fibrosis (CF) sinus disease have developmental sinus abnormalities with airway bacterial infection, inflammation, impaired mucociliary clearance and thick obstructive mucus. The pathophysiology of airway disease in CF is not completely understood, and current treatments in CF sinus disease ameliorate symptoms but do not provide a cure.
Methods
This manuscript reviews the history of CF, its manifestations in sinus disease, and the potential impact and relationship of CF on the upper and lower airway.
Results
There is increasing evidence that CF sinus disease may affect CF lung disease, the most common cause of mortality in CF. We have been limited in treating the symptoms of advanced CF sinus disease with our current therapies.
Conclusions
Recent discoveries in the pathophysiology of CF using the CF porcine animal model and exciting treatments that address the primary gene defect that may translate into improved outcomes in CF and non‐CF sinusitis in humans.
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