Long-acting bronchodilators in cystic fibrosis.

Abstract

Over 80% of patients with cystic fibrosis (CF) have bronchodilator therapy prescribed, yet bronchodilator use in CF remains controversial. The development of long-acting beta-agonist drugs for clinical use has provided additional rationale for considering bronchodilator therapy in CF. This paper will review recent developments in bronchodilator use in CF patients, with emphasis on the long-acting beta agonists. It is reported that 50 to 60% of CF patients demonstrate significant intermittent airway hyperreactivity in response to bronchodilators or challenges. The beta-agonist drugs are the most commonly prescribed bronchodilators. Several mechanisms may be implicated in therapeutic response of CF patients to bronchodilators including direct smooth muscle relaxation, increased mucociliary clearance, direct effects on inflammatory cells and bacterial adherence, and possible direct effects on CF transmembrane conductance regulator (CFTR) function. Several recent studies have shown improved outcomes with long-acting bronchodilators. In spite of the widespread use of bronchodilators, there are very few long-term studies of their effects in CF patients. However, there are clearly clinical benefits in certain situations. Further research into the most appropriate utilization of these medications to improve outcomes in patients with CF would be helpful.