title: Pulmonary exacerbations in cystic fibrosis.
abstract: OBJECTIVE
  The chronic infection and inflammation of cystic fibrosis (CF) lung disease causes a progressive decline of lung function resulting in daily symptoms such as cough and sputum production. There are intermittent episodes of acute worsening of symptoms, more commonly referred to as pulmonary exacerbations. Despite this being a common event, there is still no standardized definition of an exacerbation. A recent set of guidelines from the CF Foundation Pulmonary Therapies Committee on the treatment of exacerbations noted the paucity of data supporting commonly used therapies. This review describes our current understanding of pulmonary exacerbations and the therapies used to treat them.
  
  
  RESULTS
  The treatment of an exacerbation is intended to resolve the worsened symptoms and to restore the lung function that is commonly lost in the acute presentation. A most striking finding is the observation that for many patients there is no restoration of lung function, suggesting we either need better therapies to prevent exacerbations or better treatment of exacerbations.
  
  
  CONCLUSIONS
  We have established recommendations on specific treatment of a pulmonary exacerbation and have outlined the areas where we need better information on appropriate therapies. Once we have a standardized definition of an exacerbation, we can proceed with clinical trials of therapies specific for its treatment.
authors: Department of Medicine, Medical University of South Carolina, Charleston, South Carolina 29424, USA. stenbit@musc.edu
authors: Antine E
authors: AE
authors: Stenbit
authors: Patrick A
authors: PA
authors: Flume
mesh_terms: D003550:Cystic Fibrosis / Q000150:complications* / Q000188:drug therapy / Q000503:physiopathology; D018450:Disease Progression; D006469:Hemoptysis / Q000188:drug therapy / Q000209:etiology* / Q000503:physiopathology; D006801:Humans; D008171:Lung Diseases / Q000188:drug therapy / Q000209:etiology* / Q000503:physiopathology; D017410:Practice Guidelines as Topic