Update on pathogenesis of cystic fibrosis lung disease

Curr Opin Pulm Med. 2003 Nov;9(6):486-91. doi: 10.1097/00063198-200311000-00007.


Purpose of review: It has been an ongoing challenge to translate knowledge pertaining to the molecular basis of cystic fibrosis (CF) into a clear understanding of the development of CF lung disease. Various hypotheses have attempted to explain the apparent breach of innate defenses in CF, although a definitive explanation has been elusive.

Recent findings: Recent data suggest that altered ion transport functions--namely sodium hyperabsorption and reduced chloride secretion--lead to a depletion of airway surface liquid. As a result, the overlying mucus layer may encroach upon cell surfaces and become adherent, thus interfering with cilia-dependent and cough clearance. These static, and ultimately anaerobic, niches provide a favorable environment for the development of bacterial biofilms and persistent infection with Pseudomonas aeruginosa.

Summary: With a better understanding of pathogenic steps leading to CF lung disease, we may now be able to direct the development of therapies that will substantially improve disease outcomes.

Publication types

  • Review

MeSH terms

  • Cystic Fibrosis / genetics
  • Cystic Fibrosis / physiopathology*
  • Cystic Fibrosis Transmembrane Conductance Regulator / genetics
  • Cystic Fibrosis Transmembrane Conductance Regulator / physiology*
  • Humans
  • Ion Transport / physiology*
  • Mucus / physiology
  • Respiratory Mucosa / physiopathology


  • CFTR protein, human
  • Cystic Fibrosis Transmembrane Conductance Regulator