Bronchiectasis

Question 1

What is bronchiectasis?

Answer 1

Chronic lung inflammation causing scarring and dilation

Bronchiectasis = obstructive

*this is because the inflammation causes mucus plugs to form in the airways and this obstructs airflow

Question 2

Which disease are known to cause bronchiectasis?

Answer 2

1. Primary ciliary dyskinesia - cilia dont move and mucus gets stuck in aiways, bacteria within mucus multiply and cause pneumonia and chronic inflammation
2. Cystic fibrosis - thick sticky mucus accumulates and leads to chronic inflammation
3. Airway obstruction - tumours inside or outside airways or foreign body, prevents mucous clearance and leads to chronic inflammation

**A cause is only found in 50% of cases

Question 3

Pathology of bronchiectasis

Answer 3

Inflammatio occurs, lung function declines and hypoxia occurs as arterioles start to constrict to prevent blood flow to under-perfused areas

If inflammation is widespread, widespread constriction occurs and leads to pulmonary vascular resistance

Pulmonary hypertension

Right ventricular hypertrophy

Question 4

What are the symptoms of bronchiectasis?

Answer 4

• Wheeze and cough
• SOB
• Foul smelling mucous
• Long term clubbing (especially in cystic fibrosis)
• Coarse inspiratory crackles on inspiration
• Fever and systemic upset

Question 5

Complications of bronchiectasis

Answer 5

• Pneumonia
• Empyema
• Pneumothorax
• Meningitis
• Amyloid formation

Question 6

Investigations for bronchiectasis

Answer 6

• Radiology: chest x-ray may show bronchial wall thickening and cystic spaces if advanced
• High resolution CT: investigation of choice to detect bronchial wall thickening
• Sputum: during infective exacerbation for focused antibiotic treatment
• Test for cystic fibrosis
• Spirometry: obstructive picture

Question 7

Treatment of bronchiectasis

Answer 7

a) Airway clearance - devices to clear mucous and nebulised hypertonic saline
b) Anti-inflammatories - azithromycin has an immunomodulatory effect and reduces exacerbation frequency. Inhaled corticosteroids
c) Treat infection - pseudomonas aeruginosa only responds to quinolones
d) Treat complications - pulmonary rehab, oxygen if resp. failure, surgery for localised disease

Question 8

Investigation of choice in bronchiectasis?

Answer 8

High resolution CT scan

• Tram track, non tapering airways, signet ring sign (dilated bronchus and accompanying pulmonary artery seen in cross section)

Question 9

Regarding bronchiectasis, what factors are associated with a worse outcome?

Answer 9

Low FEV1 and infection with pseudomonas aeruginosa

Question 10

What is Kartagener’s syndrome?

Answer 10

Autosomal recessive ciliary syndrome which is characterised by primary ciliary dyskinesia and situs inversus (organs on opposite side of body)

= Primary ciliary dyskinesia + situs inversus

Question 11

What are the most common organisms associated with bronchiectasis?

Answer 11

Haemophilus influenzae (most common)

Pseudomonas aeruginosa

Klebsiella spp.

Streptococcus pneumoniae