Bronchiectasis

Chronic respiratory disease

Aetiology

• Cystic fibrosis
• ⍺1-antitrypsin deficiency
• Primary ciliary syndrome (Kartagener syndrome)
• Bronchial obstruction from foreign body, extrinsic lymph nodes compressing the bronchus, neoplasm, or mucous plug

Pathophysiology

• Structural changes - bronchial dilation, bronchial wall thickening, mucus plugging, small airways disease, emphysema
  • Most have airflow obstruction, but other patterns possible
• Chronically infected with Haemophilus influenzae and Pseudomonas aeruginosa, and sometimes Moraxella catarrhalis, Staph aureus, and Enterobactericeae
  • Persistent isolation of these organism is associated with increased frequency of exacerbations, worse quality of life, and increased mortality
• Chronic infection stimulates and sustains lung inflammation
  • Predominately neutrophilic
  • Leads to degradation of airway elastin, among other mechanisms
• Mucociliary clearance is impaired by structural bronchiectasis, airway dehydration, excess mucus volume and viscosity

Presentation

• Clinical syndrome of cough, sputum production and bronchial infection
• Exacerbations:
  • Increased airways and systemic inflammation
  • Progressive lung damage
  • Lung function decline
  • Mortality

Imaging

• Abnormal and permanent bronchial dilation

Treatment

• Treat airflow obstruction - bronchodilators
• Improve exercise capacity - pulmonary rehab
• Remove poorly functioning or diseased lung - surgical resection
  • Indications
    • Localised disease with high exacerbation frequency despite optimisation of care
    • Massive haemoptysis refractory to bronchial artery embolization
    • Bilateral bronchiectasis is not an ABSOLUTE contraindication to surgery
  • Prevents contamination of adjacent lung zones
  • Lobectomy is surgery of choice, preferably via VATS