Non-CF bronchiectasis: Orphan disease no longer

Jaafer Saadi Imam, Alexander G. Duarte

Research output: Contribution to journalReview article

1 Scopus citations

Abstract

Bronchiectasis is a complex, chronic respiratory condition, characterized by frequent cough and exertional dyspnea due to a range of conditions that include inherited mucociliary defects, inhalational airway injury, immunodeficiency states and prior respiratory infections. For years, bronchiectasis was classified as either being caused by cystic fibrosis or non-cystic fibrosis. Non-cystic fibrosis bronchiectasis, once considered an orphan disease, is more prevalent worldwide in part due to greater availability of chest computed tomographic imaging. Identification of the cause of non-cystic fibrosis bronchiectasis with the use of chest imaging, laboratory testing, and microbiologic assessment of airway secretions can lead to initiation of specific therapies aimed at slowing disease progression. Nonpharmacologic therapies such as airway clearance techniques and pulmonary rehabilitation improve patient symptoms. Inhaled corticosteroids should not be routinely prescribed unless concomitant asthma or COPD is present. Inhaled antibiotics prescribed to individuals with >3 exacerbations per year are well tolerated, reduce airway bacteria load and may reduce the frequency of exacerbations. Likewise, chronic macrolide therapy reduces the frequency of exacerbations. Medical therapies for cystic fibrosis bronchiectasis may not be effective in treatment of non-cystic fibrosis bronchiectasis.

Original languageEnglish (US)
Article number105940
JournalRespiratory Medicine
Volume166
DOIs
StatePublished - May 2020

Keywords

  • Bronchiectasis
  • Chronic pulmonary infection
  • Cystic fibrosis
  • Non-CF bronchiectasis
  • Non-cystic fibrosis bronchiectasis

ASJC Scopus subject areas

  • Pulmonary and Respiratory Medicine

Fingerprint Dive into the research topics of 'Non-CF bronchiectasis: Orphan disease no longer'. Together they form a unique fingerprint.

  • Cite this