Respiratory System >
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