Cystic fibrosis-related bone disease

Curr Opin Pulm Med. 2013 Nov;19(6):681-6. doi: 10.1097/MCP.0b013e3283659e35.


Purpose of review: This review highlights recently published data on the pathophysiology, guidelines and treatment of cystic fibrosis (CF)-related bone disease.

Recent findings: The exact role of the cystic fibrosis transmembrane conductance regulator (CFTR), specifically the ΔF508 allele, has been investigated in F508del-CFTR homozygous mice and the F508del-CFTR mutation may contribute to CF-related bone disease by slowing new bone formation. The European Cystic Fibrosis Society has issued guidelines for bone mineral density assessment, management of low-trauma fractures and bisphosphonate therapy. A systematic review based on meta-analyses reports that oral and intravenous bisphosphonates both improve bone mineral density in CF patients, but no data are available concerning the reduction of low-trauma fractures.

Summary: European Cystic Fibrosis Society guidelines may help physicians to improve the management of CF-related bone disease.

Publication types

  • Review
  • Systematic Review

MeSH terms

  • Animals
  • Bone Density / drug effects*
  • Bone Density / genetics
  • Bone Density Conservation Agents / therapeutic use*
  • Bone Diseases / drug therapy
  • Bone Diseases / genetics
  • Bone Diseases / physiopathology*
  • Bone Diseases / prevention & control*
  • Cystic Fibrosis / drug therapy
  • Cystic Fibrosis / genetics
  • Cystic Fibrosis / physiopathology*
  • Cystic Fibrosis Transmembrane Conductance Regulator / genetics
  • Diphosphonates / therapeutic use*
  • Female
  • Fractures, Bone / physiopathology
  • Fractures, Bone / prevention & control
  • Humans
  • Male
  • Mice
  • Mice, Inbred CFTR
  • Mutation / genetics
  • Practice Guidelines as Topic
  • Risk Factors
  • Vitamin D / therapeutic use*


  • Bone Density Conservation Agents
  • CFTR protein, human
  • Diphosphonates
  • Cystic Fibrosis Transmembrane Conductance Regulator
  • Vitamin D