Medical reversal of chronic sinusitis in a cystic fibrosis patient with ivacaftor

Eugene H. Chang, Xiao Xiao Tang, Viral S. Shah, Janice L. Launspach, Sarah E. Ernst, Brieanna Hilkin, Philip H. Karp, Mahmoud H. Abou Alaiwa, Scott M. Graham, Douglas B. Hornick, Michael J. Welsh, David A. Stoltz, Joseph Zabner

Research output: Contribution to journalArticlepeer-review

29 Scopus citations

Abstract

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.

Original languageEnglish (US)
Pages (from-to)178-181
Number of pages4
JournalInternational Forum of Allergy and Rhinology
Volume5
Issue number2
DOIs
StatePublished - Feb 1 2015
Externally publishedYes

Keywords

  • ASL
  • Airway surface liquid
  • CF
  • CFTR
  • Cystic fibrosis
  • Ivacaftor

ASJC Scopus subject areas

  • Immunology and Allergy
  • Otorhinolaryngology

Fingerprint

Dive into the research topics of 'Medical reversal of chronic sinusitis in a cystic fibrosis patient with ivacaftor'. Together they form a unique fingerprint.

Cite this