Medical reversal of chronic sinusitis in a cystic fibrosis patient with ivacaftor
EH Chang, XX Tang, VS Shah… - International forum of …, 2015 - Wiley Online Library
EH Chang, XX Tang, VS Shah, JL Launspach, SE Ernst, B Hilkin, PH Karp, MH Abou Alaiwa…
International forum of allergy & rhinology, 2015•Wiley Online LibraryBackground Chronic sinusitis is universal in cystic fibrosis (CF) and our current treatments
are ineffective in reversing sinus disease. The objective of this work was to determine if
increasing CF transmembrane conductance regulator (CFTR) activity by ivacaftor could treat
CF sinus disease and assess its effect on primary sinus epithelial cultures. Methods Case
report of 1 patient with long‐standing chronic sinus disease and a new diagnosis of CF with
a mild mutation (P205S) and a severe mutation (G551D). We discuss clinical changes in …
are ineffective in reversing sinus disease. The objective of this work was to determine if
increasing CF transmembrane conductance regulator (CFTR) activity by ivacaftor could treat
CF sinus disease and assess its effect on primary sinus epithelial cultures. Methods Case
report of 1 patient with long‐standing chronic sinus disease and a new diagnosis of CF with
a mild mutation (P205S) and a severe mutation (G551D). We discuss clinical changes in …
Background
Chronic sinusitis is universal in cystic fibrosis (CF) and our current treatments are ineffective in reversing sinus disease. The objective of this work was to determine if increasing CF transmembrane conductance regulator (CFTR) activity by ivacaftor could treat CF sinus disease and assess its effect on primary sinus epithelial cultures.
Methods
Case report of 1 patient with long‐standing chronic sinus disease and a new diagnosis of CF with a mild mutation (P205S) and a severe mutation (G551D). We discuss clinical changes in symptoms, radiographic findings, nasal potential difference testing, and nasal pH values before and after treatment with ivacaftor. We then developed primary sinonasal epithelial cell cultures from a biopsy of the patient to determine changes in airway surface liquid (ASL) pH and ASL viscosity after ivacaftor treatment.
Results
Ivacaftor treatment reversed CT findings of CF sinus disease, increased nasal voltage and pH, and resolved sinus symptoms after 10 months of therapy. Ivacaftor significantly increased ASL pH and decreased ASL viscosity in primary airway cultures.
Conclusion
This report documents the reversal of CF sinus disease. Based on our in vivo and in vitro results, we speculate that ivacaftor may reverse CF sinusitis by increasing ASL pH and decreasing ASL viscosity. These studies suggest that CFTR modulation may be effective in treating CF and perhaps non‐CF sinusitis.
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