Bronchiectasis

Abnormal dilation of distal airways, associated with chronic cough and excessive sputum production. 

Causes

Genetic	CF, Young’s syndrome, Kartagener’s syndrome, yellow nail syndrome, α1-antitrypsin deficiency
Mechanical	Malignancy, foreign body
Childhood infection	Pertussis, measles, TB, pneumonia
Immunological	Allergic bronchopulmonary aspergillosis, hypogammaglobulinaemia, HIV, leukaemia, autoimmune

Clinical features

Symptoms

• Chronic productive cough
• Haemoptysis
• Recurrent chest infections

Signs of bronchiectasis

• Clubbing
• Inspiratory clicks
• Coarse inspiratory crepitations 
• Large airway rhonchi

Signs of cause

• Curved yellow nails and lymphoedema (yellow nail syndrome)
• Dextrocardia (Kartagener’s syndrome)
• Young and thin (CF)

Investigations

• Diagnosis
  • Chest X-ray: tramlines and ring shadows
  • High-resolution CT chest: ‘signet ring sign’ (bronchi larger than the adjacent vasculature) and bronchial wall thickening – diagnostic test
  • Spirometry: variable but often obstructive pattern
• To ascertain cause
  • Immunoglobulins and electrophoresis
  • Aspergillus precipitins and serum IgE 
  • CF sweat test/genetic mutation analysis
  • HIV
  • Rheumatoid factor
  • α1-antitrypsin
• Sputum culture (to determine any colonising bacteria and sensitivities)

Management

• Exercise and improved nutrition
• Chest physiotherapy
• Prompt treatment of infections (choose antibiotics based on culture sensitivities)
• Rotating antibiotics may be used for prophylaxis if recurrent infections
• Trial of bronchodilator and nebulised hypertonic saline
• Pneumococcal/influenza vaccines
• Lung resection may be considered in localised disease with a poor response to medical management