What is bronchiectasis?
Permenant dilation of the airways, due to desctruction of the elastic and muscular tissue of the bronchial walls.
Outline the pathophysiology of bronchiectasis.
- Infection – bacterial colonisation of the tissue leads to dilation and fibrosis of airways
- Inflammation leads to increased mucus production, bronchial wall oedema, release of cytokines
- Damage to airways predisposes to further colonisation (vicious cycle)
What are some common causes of bronchiectasis?
POST INFECTIOUS: TB, pneumonia, whooping cough
GENETIC: Cystic fibrosis, alpha-1 anti-trypsin deficiency, Kartagener’s syndrome, connective tissue disorders
OBSTRUCTION: tumour e.g. lymphadeonpathy causing external compression, foreign body, broncholith
OTHER: Inflammatory diseases e.g. IBD, RhA, primary ciliary dyskinesia, allergc bronchopulmonary aspergillosis
What is alpha-1 anti-trypsin deficiency?
Congenital disorder
Alpha-1 antryptase is a protease inhibitor, produced in liver.
Deficiency allow breakdown of elastin by neutrophils
Symptoms of bronchiectasis
Cough, with sputum (purulent or, in 50%, haemoptysis), dyspnoea, fever, weight loss
Symptoms come on after an acute resp illness
Signs of bronchiectasis
INSPECTION: clubbing
AUSCULATION: wheeze, rhonchi, high-pitched inspiratory squeaks, corase crackles at bases which shift with coughing
Investigations for bronchiectasis
FBC - leukocytosis
Sputum - culture and sensitivity
CXR - dilated bronchi with tramline shadows
High-res chest CT - dilated bronchi and thickened walls
Which is the best diagnostic method for bronchiectasis?
High-res chest CT
Management of bronchiectasis
- ANTIBIOTICS - x2, one against Pseudomonas - this can be oral cipro. May need prophylaxis in >3 exacerbations/year
- STEROIDS - inhaled e.g. fluticasone
- PHYSIO - sputum and mucus clearance
- BRONCHODILATORS - if pt has responsive disease
- MAINTAIN HYDRATION
- SURGERY - if necessary - resection or lung transplant
Complications of bronchiectasis
LIFE-THREATENING HAEMOPTYSIS CAN CAUSE BRONCHIAL ARTERY EMBOLISATION
Pleural effusion, empyema, pneumothorax, cor pulmonale, amyloidosis
What is cor pulmonale?
LUNGS CAUSE HEART TO FAIL.
RV enlargement and failure due to:
- increased pressure in lungs
- increased vascular resistance e.g. pulmonic stenosis
