Pathophysiology and management of pulmonary infections in cystic fibrosis

Am J Respir Crit Care Med. 2003 Oct 15;168(8):918-51. doi: 10.1164/rccm.200304-505SO.


This comprehensive State of the Art review summarizes the current published knowledge base regarding the pathophysiology and microbiology of pulmonary disease in cystic fibrosis (CF). The molecular basis of CF lung disease including the impact of defective cystic fibrosis transmembrane regulator (CFTR) protein function on airway physiology, mucociliary clearance, and establishment of Pseudomonas aeruginosa infection is described. An extensive review of the microbiology of CF lung disease with particular reference to infection with P. aeruginosa is provided. Other pathogens commonly associated with CF lung disease including Staphylococcal aureus, Burkholderia cepacia, Stenotrophomonas maltophilia, Achromobacter xylosoxidans and atypical mycobacteria are also described. Clinical presentation and assessment of CF lung disease including diagnostic microbiology and other measures of pulmonary health are reviewed. Current recommendations for management of CF lung disease are provided. An extensive review of antipseudomonal therapies in the settings of treatment for early P. aeruginosa infection, maintenance for patients with chronic P. aeruginosa infection, and treatment of exacerbation in pulmonary symptoms, as well as antibiotic therapies for other CF respiratory pathogens, are included. In addition, the article discusses infection control policies, therapies to optimize airway clearance and reduce inflammation, and potential future therapies.

Publication types

  • Review

MeSH terms

  • Achromobacter
  • Ambulatory Care / methods
  • Burkholderia Infections / drug therapy
  • Burkholderia cepacia
  • Chronic Disease
  • Cystic Fibrosis / complications*
  • Cystic Fibrosis / diagnosis
  • Cystic Fibrosis / epidemiology
  • Cystic Fibrosis / genetics
  • Cystic Fibrosis Transmembrane Conductance Regulator / genetics
  • Drug Resistance, Bacterial
  • Gene Transfer Techniques / trends
  • Gram-Negative Bacterial Infections / drug therapy
  • Humans
  • Infection Control / methods
  • Mucociliary Clearance
  • Mycobacterium Infections, Nontuberculous / drug therapy
  • Pseudomonas Infections / diagnosis
  • Pseudomonas Infections / drug therapy*
  • Pseudomonas Infections / etiology*
  • Pseudomonas Infections / physiopathology
  • Respiratory Tract Infections / diagnosis
  • Respiratory Tract Infections / drug therapy*
  • Respiratory Tract Infections / etiology*
  • Respiratory Tract Infections / physiopathology
  • Stenotrophomonas maltophilia
  • Survival Analysis
  • Treatment Outcome
  • United States / epidemiology


  • CFTR protein, human
  • Cystic Fibrosis Transmembrane Conductance Regulator