Early lung disease in cystic fibrosis

Lancet Respir Med. 2013 Apr;1(2):148-57. doi: 10.1016/S2213-2600(13)70026-2. Epub 2013 Mar 12.


Lung disease in patients with cystic fibrosis is characterised by inflammation and recurrent and chronic infections leading to progressive loss in pulmonary function and respiratory failure. Early management of disease results in substantially improved pulmonary function at first testing (at roughly 6 years of age), but the annual decline in pulmonary function tests in older patients has remained unchanged showing how important the early years are in the disease process. Treatment regimens for patients with cystic fibrosis have changed from predominantly symptomatic treatment to preventive or causal (ie, treatments that address the underlying mechanisms of disease) therapeutic interventions. The infant and preschool age (2-5 years) could represent a unique period of opportunity to postpone or even prevent the onset of cystic fibrosis lung disease. We summarise the current knowledge and the methods used to characterise and quantify early lung disease. We discuss treatment strategies including new drugs that are being developed and their potential role in the treatment of early lung disease in patients with cystic fibrosis.

Publication types

  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Adult
  • Airway Remodeling / drug effects
  • Anti-Bacterial Agents / therapeutic use
  • Child
  • Child, Preschool
  • Clinical Trials as Topic
  • Cystic Fibrosis* / complications
  • Cystic Fibrosis* / diagnosis
  • Cystic Fibrosis* / physiopathology
  • Cystic Fibrosis* / therapy
  • Disease Management
  • Disease Progression
  • Drugs, Investigational / pharmacology
  • Early Medical Intervention / methods
  • Humans
  • Infant
  • Inflammation* / diagnosis
  • Inflammation* / drug therapy
  • Inflammation* / etiology
  • Lung Diseases* / drug therapy
  • Lung Diseases* / etiology
  • Lung Diseases* / physiopathology
  • Respiratory Function Tests / methods
  • Respiratory System Agents / pharmacology*
  • Respiratory Tract Infections* / diagnosis
  • Respiratory Tract Infections* / drug therapy
  • Respiratory Tract Infections* / etiology


  • Anti-Bacterial Agents
  • Drugs, Investigational
  • Respiratory System Agents