title: Ibuprofen therapy for cystic fibrosis lung disease: revisited.
abstract: OBJECTIVE
  Ibuprofen was first advocated as a chronic therapy for cystic fibrosis lung disease in 1995, following a favorable report of a 4-year controlled trial. However, clinical use has been limited primarily by the concern about adverse effects. Additional clinical studies were advocated to better assess the risk-benefit profile. The results of several studies have been published within the last couple of years.
  
  
  RESULTS
  Results from a 2-year multicenter trial were consistent with the 4-year trial in demonstrating a beneficial effect of ibuprofen on lung function in children with mild to moderate lung disease. The drug also showed a favorable safety profile. Results from an analysis of observational data from the Cystic Fibrosis Foundation Patient Registry collected over 7 years revealed that 'real world' clinical use of ibuprofen was also associated with a beneficial effect. The occurrence of gastrointestinal bleeding was higher in those treated with ibuprofen, but the incidence was very low.
  
  
  CONCLUSIONS
  Ibuprofen has now been shown in two long-term clinical trials to slow disease progression, with real-world clinical use supporting its effectiveness. Although the therapy is not without adverse effects, the benefits appear to outweigh the risks.
authors.affiliation: Department of Pediatrics, Case Western Reserve University School of Medicine and Rainbow Babies and Children's Hospital, Cleveland, Ohio 44106, USA. michael.konstan@case.edu
authors.forename: Michael W
authors.initials: MW
authors.lastname: Konstan
mesh_terms: D000293:Adolescent; D000894:Anti-Inflammatory Agents, Non-Steroidal / Q000009:adverse effects / Q000627:therapeutic use*; D002648:Child; D002675:Child, Preschool; D003550:Cystic Fibrosis / Q000188:drug therapy*; D018450:Disease Progression; D006471:Gastrointestinal Hemorrhage / Q000139:chemically induced / Q000453:epidemiology; D006801:Humans; D007052:Ibuprofen / Q000009:adverse effects / Q000627:therapeutic use*; D015994:Incidence; D018570:Risk Assessment
chemical_list: D000894:Anti-Inflammatory Agents, Non-Steroidal; D007052:Ibuprofen