Bronchiectasis

Definition

• Permanent dilatation of the small airways, causing persistent microbial infection

Etiology

• Focal
  • Acquired
    • Chronic infection
      • Granulomatous
        • Tuberculosis
        • Histoplasmosis
        • MAC/NTM
      • Bacterial: S. aureus, Klebsiella, Pseudomonas
      • Viral
    • Obstruction
      • Foreign body
      • Tumour
    • Right middle lobe syndrome
    • Swyer-James-MacLeod syndrome (postinfective obliterative bronchiolitis)
  • Congenital
    • Bronchopulmonary sequestration
    • Williams-Campbell syndrome (cartilage deficiency)
    • Mouner-Kuhn syndrome (tracheobronchmegaly)
• Diffuse
  • Genetic
    • Cystic fibrosis (CF)
    • Primary ciliary dyskinesia/Kartagener's syndrome
    • Alpha-1 antitrypsin deficiency
    • Young's syndrome
  • Infectious: Non-tuberculous mycobacterium/MAC
  • Immunodeficiency: Hypogammaglobulinemia
  • Inflammatory: Allergic bronchopulmonary aspergillosis (ABPA)
  • Autoimmune or immune-mediated
    • Rheumatoid arthritis
    • Sjögren's syndrome
    • Inflammatory bowel disease
  • Bronchiolitis obliterans after transplant
  • Recurrent aspiration
  • Near drowning
  • Toxic inhalation
  • Miscellaneous
    • Yellow nail syndrome
    • Postradiation traction bronchiectasis
    • IPF

Clinical Manifestations

History

• Chronic cough (98%)
• Daily sputum (78%)
• Rhinosinusitis (73%)
• Dyspnea (62%)
• Hemoptysis (27%)
• Recurrent pleurisy (20%)

Investigations

• Labs
  • CBC, immunoglobulins, and HIV testing to rule out immunodeficiency
  • Sputum culture for bacteria, mycobacteria, and fungi; consider BAL
  • Sweat chloride testing +/- genetic testing to rule out CF
  • Alpha-1 antitrypsin levels (<11 in deficiency)
  • Nasal brush/biopsy to rule out ciliary dyskinesia
• Imaging
  • High-resolution CT
    • Signet-ring sign (airway >1.5x blood vessel) is best sign
    • Tram-tracking
• Other
  • PFTs
  • Bronchoscopy
  • Swallowing assessment to rule out aspiration

Management

Acute exacerbation

• Send sputum cultures every time
• Cover empirically with usual pneumonia treatment x 14 days
• Add Pseudomonas or MRSA coverage if needed
• Consider prednisone if asthma or ABPA

Chronic

• Chest physiotherapy it most important
• Mucous (questionable utility outside of CF)
  • Hypertonic saline
  • Mannitol
  • DNAse/Pulmozyme is BAD outside of CF
  • Mucomyst
• Antiinflammatory
  • Consider inhaled bronchodilators if responsive
  • Consider azithromycin 500mg po MWF
• Inhaled antimicrobials, if colonozed with Pseudomonas and more than 3 exacerbations annually
  • Inhaled tobramycin
  • Inhaled colistin
  • Possibly inhaled ciprofloxacin
• Surgery: resection or transplantation can sometimes be considered
• Supportive
  • Annual flu vaccine
  • Smoking cessation
  • Pulmonary rehabilitation
  • GERD treatment