Bronchiectasis

“Abnormal and permanently dilated airways that result in a build-up of excess mucus, increasing risk of infections”

Risk Factors

• Cystic fibrosis
• Pulmonary disorders (e.g. TB, COPD, IPF)
• Exposure to toxic fumes or chemicals
• Childhood respiratory infections
• Allergic bronchopulmonary aspergillosis
• Immune deficiency

Aetiology

• Cystic fibrosis
• Mechanical bronchial obstruction (e.g. tumour, mucus, foreign body)
• Post-infective bronchial damage
• Granuloma (TB/ sarcoidosis)
• Immune deficiency
• Impaired mucociliary clearance

Pathophysiology

• Cycle of initial infection → inflammation (cytokines and neutrophil elastases) → impaired mucociliary clearance → hypersecretion of mucus (mucus plug) → airway obstruction → microbial colonisation → bronchial dilatation and airway destruction → further inflammation

Clinical Presentation

• Persistent cough
• Copious production of purulent sputum
• Breathlessness (later stages of disease)
• Haemoptysis
• Infection
  • An increased volume of sputum and purulence
  • Fever
  • Systemic upset
• Pleuritic chest pain

• O/E
  • Can be normal
  • Coarse crackles heard on auscultation
  • Clubbing (especially when due to CF)

Investigations

• HRCT scanning
  • Non-tapering ‘tram track’ airways
  • Signet ring sign (increased broncho-arterial ratio)
• Chest X ray
  • Often normal
  • ‘Tram track’ airways
  • Ring shadows
  • Cysts
• Sputum sample
• Immune assessment – immunoglobulins and responses to Hib; tetanus and pneumococcal vaccines

Management

• Airway clearance
  • Breathing technique
  • Autogenic (self-)drainage
  • Postural drainage
  • Nebulised hypertonic saline
• Anti-inflammatories
  • Long-term azithromycin (macrolide)
  • Inhaled corticosteroids
• Treatment of infection (2 weeks)
  • Pseudomonas – 750mg ciprofloxacin BD
  • Haemophilus influenzae – amoxicillin/ co-amoxiclav/ doxycycline
  • IV cephalosporin for multi-resistant species
• Treatment of complications
  • Breathlessness and reduced exercise capacity – pulmonary rehabilitation
  • Massive haemoptysis – resuscitation and airway protection → bronchial artery embolization
  • Respiratory failure – oxygen and NIV
  • Localised disease – surgery

Complications

• Breathlessness
• Reduced exercise capacity
• Massive haemoptysis
• Respiratory failure
• Localised disease