Cystic fibrosis pulmonary guidelines: Chronic medications for maintenance of lung health

Peter J. Mogayzel, Edward T. Naureckas, Karen A. Robinson, Gary Mueller, Denis Hadjiliadis, Jeffrey B. Hoag, Lisa Lubsch, Leslie Hazle, Kathy Sabadosa, Bruce Marshall

Research output: Contribution to journalReview articlepeer-review

367 Scopus citations


Rationale: Cystic fibrosis (CF) is an autosomal recessive disease characterized by abnormal airways secretions, chronic endobronchial infection, and progressive airway obstruction. The use ofmedications to slowtheprogression of lungdiseasehas ledtosignificant improvement in survival. An evidence review of chronic medications for CF lung disease was performed in 2007 to provide guidance to clinicians in evaluating and selecting appropriate treatment for individuals with this disease. We have undertaken a new review of the literature to update the recommendations, including consideration of new medications and additional evidence on previously reviewed therapies. Amultidisciplinary committee of experts in CF pulmonary care was established to review the evidence for use of chronic medications for CF lung disease and make treatment recommendations. Published evidence for chronic lung therapies was systematically reviewed and resulting treatment recommendationswere graded based on theUnited States Preventive Services Task Force scheme. These guidelines provide up-to-date evidence of safety and efficacy of chronic treatments of CF lung disease, including the use of novel therapies that have not previously been included in CF pulmonary guidelines.

Original languageEnglish (US)
Pages (from-to)680-689
Number of pages10
JournalAmerican journal of respiratory and critical care medicine
Issue number7
StatePublished - Apr 1 2013


  • Antibiotics
  • Antiinflammatory agents
  • Bronchodilators
  • CFTR modulators
  • Hypertonic saline

ASJC Scopus subject areas

  • Pulmonary and Respiratory Medicine
  • Critical Care and Intensive Care Medicine


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