Ivacaftor and sinonasal pathology in a cystic fibrosis patient with genotype deltaF508/S1215N

J Cyst Fibros. 2015 May;14(3):412-3. doi: 10.1016/j.jcf.2014.07.013. Epub 2014 Aug 26.


In patients with Cystic Fibrosis and a type III mutation, ivacaftor (Kalydeco(®), Vertex) can increase the opening time of the CFTR channel and improve chloride transport. Research showed significant improvement of lung function and increase in weight following ivacaftor use. However, ivacaftor showed to have adverse events on the sinonasal system as well, such as upper respiratory tract infections, nasal congestion and headaches. This case report showed a positive effect of ivacaftor on the sinonasal pathology in a 17 year old patient with CF. After 5 months of ivacaftor use, the CT-sinus showed complete resolution of the opacification of the paranasal sinuses and a decrease in symptoms of sinonasal disease. This positive effect of ivacaftor on sinonasal pathology seems promising, therefore more research is needed to evaluate the effect of ivacaftor on the upper airways in CF.

Keywords: CFTR potentiator; Computed Tomography; Cystic Fibrosis; Ivacaftor; Rhinosinusitis.

Publication types

  • Case Reports

MeSH terms

  • Adolescent
  • Aminophenols / administration & dosage*
  • Cystic Fibrosis / drug therapy*
  • Cystic Fibrosis / genetics
  • Cystic Fibrosis / physiopathology
  • Cystic Fibrosis Transmembrane Conductance Regulator / genetics
  • Cystic Fibrosis Transmembrane Conductance Regulator / metabolism
  • DNA / genetics
  • Female
  • Forced Expiratory Volume
  • Genotype
  • Humans
  • Ion Transport / genetics
  • Mutation
  • Paranasal Sinus Diseases / complications
  • Paranasal Sinus Diseases / diagnostic imaging
  • Paranasal Sinus Diseases / drug therapy*
  • Paranasal Sinuses / diagnostic imaging*
  • Quinolones / administration & dosage*
  • Tomography, X-Ray Computed


  • Aminophenols
  • Quinolones
  • Cystic Fibrosis Transmembrane Conductance Regulator
  • ivacaftor
  • DNA