A pilot study of zafirlukast as an anti-inflammatory agent in the treatment of adults with cystic fibrosis

J Cyst Fibros. 2003 Mar;2(1):25-8. doi: 10.1016/S1569-1993(02)00142-X.


Background: Persistent endobronchial inflammation is in part responsible for the attrition of lung function seen in cystic fibrosis. Leukotrienes act as pro-inflammatory mediators. The aim of this study was to assess the efficacy of the leukotriene receptor antagonist zafirlukast as a potential anti-inflammatory agent in the treatment of adult patients with cystic fibrosis.

Methods: Clinically stable patients were enrolled in the study if they had no history or clinical evidence of asthma, bronchial hyper-reactivity, or aspergillosis. They were randomised to receive zafirlukast 20 mg twice daily with all routine treatment for four months or routine treatment alone in an open cross-over design. Primary endpoints were changes in respiratory function tests and a modified NIH clinical score.

Results: Thirty patients were enrolled and 25 completed. There was a significant improvement in the modified NIH clinical score but no significant increase in respiratory function with zafirlukast.

Conclusions: Patients receiving a leukotriene receptor antagonist in addition to routine treatments showed significant improvement in a clinical score which is a composite of clinical wellbeing, chest radiograph appearance, and physical examination. Respiratory function showed a non-significant trend towards improvement with treatment. Zafirlukast may benefit patients with CF. An adequately powered study is justified on the basis of these results.

Publication types

  • Clinical Trial
  • Randomized Controlled Trial

MeSH terms

  • Adult
  • Anti-Inflammatory Agents / therapeutic use*
  • Cystic Fibrosis / drug therapy*
  • Female
  • Humans
  • Indoles
  • Leukotriene Antagonists / therapeutic use*
  • Male
  • Phenylcarbamates
  • Pilot Projects
  • Sulfonamides
  • Tosyl Compounds / therapeutic use*
  • Treatment Outcome


  • Anti-Inflammatory Agents
  • Indoles
  • Leukotriene Antagonists
  • Phenylcarbamates
  • Sulfonamides
  • Tosyl Compounds
  • zafirlukast