• Uncommon disease caused by chronic infection and inflammation
  • Characterized by dilation and destruction of larger bronchi
    • Mucus build up attracts bacteria/microbes--> bacteria multiply--> additional lung infection/inflammation-->more airway damage--> over time, enlarged/widened airways make it harder to breath and clear mucus
  • Commonly caused by cystic fibrosis, immune defects, recurrent pneumonias, or (less commonly), congenital or idiopathic

Clinical Features

An example of clubbing.

Differential Diagnosis

Acute dyspnea




  • Evaluate for alternative causes of symptoms
  • CXR
    • Thickening of airway walls and/or airway dilation
    • Linear perihilar densities, indistinctness of central pulmonary arteries
    • "Tram-track sign"; thickened, dilated airways perpendicular to x-ray beam
    • Elongated, tubular opacities due to mucus plugs
  • CT chest
    • Sensitive and specific
    • Airway dilation, signet ring sign (thickened, dilated airway adjacent to smaller artery
    • Lack of normal bronchial tapering-->visible medium bronchi extending out closer to pleura
    • "Tram-track" sign
  • Infectious workup
    • Consider sputum for TB
  • Diagnosis of cause generally outside ED scope but may include workup for cystic fibrosis, immunodeficiency, ciliary dysmotility


  • Bronchodilators
  • Supplemental O2 PRN
  • Chest PT
  • Mucolytics (e.g. NAC mucomyst, hypertonic saline nebs)
  • Antibiotics
    • Some patients may be on chronic macrolides or other antibiotics
    • If no CF and no prior culture data, treat against H. influenzae, M. catarrhalis, S. aureus, and S. pneumoniae (e.g. amoxicillin-clavulanate, azithromycin, TMP-SMX)
    • If CF or more severe exacerbation, broaden antibiotics, knowing that many CF patients develop highly resistant polymicrobial infections and likely require multiple antibiotics (e.g. tobramycin, aztreonam, Zosyn, cefepime
    • Consider inhaled antibiotics to reduce sputum bacterial load
  • Treat massive hemoptysis


See Also

External Links