Bronchiectasis

Question 1

Define bronchiectasis summarising its aetiology and epidemiology

Answer 1

Definition: Lung airway disease characterised by chronic bronchial dilation, impaired mucociliary clearance and frequent bacterial infections.

Aetiology/risk factors of bronchiectasis:

• Chronic lung inflammation leads to fibrosis and permanent dilation of the bronchi
• This leads to pooling of mucus, which predisposes to further cycles of infection, damage and fibrosis of bronchial walls.
• Causes of bronchiectasis:
  
  • Idiopathic (50%)
  • Post-infectious (e.g. pneumonia, whooping cough, TB)
  • Host defence defects(e.g. Kartagener’s syndrome, cystic fibrosis)
  • Obstruction of bronchi (e.g. foreign body, enlarged lymph nodes)
  • GORD
  • Inflammatory disorders (e.g. rheumatoid arthiritis)

Epidemiology:

• Most often arises initially in childhood
• Incidence has decreased with the use of antibiotics
• 1/1000 per year

Question 2

Describe the history/presenting symptoms of bronchiectasis

Answer 2

• Productive cough with purulent sputum or haemoptysis.
• Breathlessness
• Chest pain
• Malaise
• Fever
• Weight loss
• Symptoms usually begin after an acute respiratory illness.

Question 3

What are the signs of bronchiectasis upon physical examination?

Answer 3

• Clubbing
• Coarse crepitations (usually at lung bases) these shift with coughing
• Wheeze

Question 4

What investigations are used to identify bronchiectasis?

Answer 4

First investigations to order
Sputum:
- Culture and sensitivity
- Common organisms include pseudomonas aeruginosa, haemophilus influenzae, staphylococcus aureus, streptococcus pneumoniae, klebsiella, mycobacteria

CXR:

• Dilated bronchi (may be seen as parallel lines going from the hilum to the diaphragm aka tramline shadows)
• Fibrosis
• Atelectasis
• Pneumonic consolidations
• May be NORMAL

High resolution CT:

• Best diagnostic method for bronchiectasis
• Shows dilated brinchi with thickened walls

Other investigations:

• Bronchography (rarely used)
• FBC
• bronchial biopsy and electron microscopy of cilia
• Cystic fibrosis transmembrane regular (CFTR) protein gene mutation testing
• swallow study
• Sputum pH monitoring

Question 5

How is bronchiectasis managed?

Answer 5

1st line:
- Exercise and improved nutrition plus airway clearance therapy

Adjunct:
- Inhaled bronchodilator- considered in patients with responsive disease
- Inhaled Corticosteroids (e.g. fluticasone)- reduces inflammation and volume of sputum but does NOT affect the frequency of exacerbations or lung formation
-Maintain hydration and flu vaccination
- Long-term oral macrolide
with high risk for or known chronic Pseudomonas infection
- Inhaled antibiotic
with acute exacerbation: mild to moderate disease
- Short-term oral antibiotic
with acute exacerbation: severe or not responding/resistant to oral antibiotics
- Short-term intravenous antibiotic
with recurrent infections, severe haemoptysis, or focal disease (treat with TWO IV ANTIBIOTICS)
- Bronchial artery embolisation- if life-threatening haemoptysis due to bronchiectasis
- Surgery

Question 6

What are the possible complications of bronchiectasis?

Answer 6

• Life-threatening haemoptysis
• Persistent infections
• Empyema
• Respiratory failure
• Cor pulmonale
• Multi-organ abscesses

Question 7

Summarise the prognosis for patients with bronchiectasis

Answer 7

Most patients continue to have symptoms after 10 years.