New therapeutic options for noncystic fibrosis bronchiectasis.

Abstract

Patients with noncystic fibrosis bronchiectasis (NCFB) share many of the respiratory symptoms of cystic fibrosis and often are provided therapies effective in cystic fibrosis, often without clear evidence of benefit. There are currently no approved therapies for NCFB, but in recent years, there has been increased interest in developing new therapies due to the increasing prevalence and perceived unmet needs. This review is meant to provide the most recent information to clinicians about currently available and pipeline therapies for NCFB. Inhaled antibiotics may provide effective bacterial suppressive therapy with an acceptable safety profile in adults with NCFB, although evidence of improved outcomes is limited. Inhaled hyperosmolar agents such as hypertonic saline and mannitol are promising but study results have been mixed. Macrolide antibiotics have anti-inflammatory properties and, in several randomized controlled trials, demonstrated the benefit of chronic low-dose treatment. Other anti-inflammatory agents that have shown promising preliminary results include statins and neutrophil elastase inhibitors. There is high-quality evidence supporting chronic low-dose macrolide therapy in patients with NCFB. There is limited evidence of benefit of other therapies, including inhaled antibiotics and pharmacologic agents to enhance mucus clearance.