Bronchiectasis

Question 1

Bronchiectasis : Definition

Answer 1

• Chronic inflammation causes bronchi and bronchioles to get damaged and dilated
• Thick mucus plugs form which narrow the bronchi
• Thus, obstruct air flow in and out of the lungs

Question 2

Bronchiectasis : Pathophysiology

Answer 2

1. Chronic inflammation is usually secondary to impairment of the mucociliairy escalator
2. Mucus traps inhaled pathogens and cilia moves it up the oesophagus to be removed via coughing
3. Damage to the cilia leaves the mucus stuck in the airways
4. Bacteria trapped in the mucus begin to multiply and causing pneumonia
5. Recurrent pneumonia leads to recurrent infections and inflammation
6. Dilation of the airways - progressive bronchial damage secondary to recurrent infections
7. Chronic inflammation further damages;
   - The cilia on the surface of the epithelial cells
   - Breaks down elastic fibres in the walls of the bronchi and alveoli
   - Fibroblasts attempt to repair damaged elastin and replace it with collagen - causing fibrosis

Result is;
Dilated, stiff airways which are clogged up with sputum that cannot be cleared

Question 3

Bronchiectasis : Causes

Answer 3

1. Immobility of cilia
   * Cystic fibrosis
   * Primary ciliary dyskinesia
   * Alpha - 1 anti trypsin deficiency
2. Ostructive disease
   * COPD
   * Asthma
3. Connective tissue disorders
   * Rheumatoid arthrits
   * Sjoren’s syndrome
4. Immunodeficiency
   * HIV

Question 4

Bronchiectasis : Clinical features

Answer 4

• Daily chronic cough with muco-purulent sputum
• Dyspnoea and fatigue - particularly on exertion
• Recurrent fever - around 50%
• Weight loss

Question 5

Bronchiectasis : Main risk factors

Answer 5

• Cystic fibrosis
• Primary ciliary dyskinesia
• Immunodeficiency
• Previous lung infections - severe viral or bacterial lung infections result in bronchial destruction and bronchiectasis

Question 6

Bronchiectasis : Clinical signs

Answer 6

Auscultation :
* Inspiratory crackles
* Inspiratory wheeze

Question 7

Bronchiectasis : Investigations

Answer 7

1. CXR : tramlines
2. CT chest - diagnostic, gold standard
   - Shows dilation of bronchi with/without airway thickening
3. FBC;
   * Raised WBC may show infective exacerbation
   * Raised eisenophils - underlying allergic bronchopulmonary aspergillosis is possible
4. Pulmonary function test
   FEV1/FVC < 70% : Obstructive disease
5. Sputum sample and sensitivity
   - Pseudomonas aureginosa (Gram - ) most common pathogen found in sputum - 25% of patients

Question 8

Bronchiectasis : Investigations to identify underlying aietiology

Answer 8

1. Serum alpha-1 antitrypsin
   * helps to identify disease if also has associated panacinar emphysema
2. Serum immunoglonbulin
   * helps to identify immunoglobulin deficiencies,
3. ** Serum HIV antibody **
   * Poor immune system can predispose development of severe lung damage and bronchiectasis
4. Rheumatoid factor
   * Test in all patients
   more common in RA patient population
   * Systemic inflammation extending to the lungs causing inflammation and ILD
   * Bronchiectasis may precede other systemic findings of rheumatoid arthritis
5. Specific IgE test for Aspergillus fumigatus
   * If occupation exposure is known

Question 9

Bronchiectasis : Management

Answer 9

1. Mainatainance Mx
   * Exercise and improved nutrition
   * Improved outcome and counteracts weights loss
2. Airway clearance therapy
   * Maintenance of oral hydration
   * Coughing/postural drainage TDS with respiratory physiotherapist
   * Use of nebulised bronchodilators and nebulised hypertonic saline
   -Helps to promote mucus clearance by inducing coughing

Question 10

Bronchiectasis : organisms causing acute exacerbations

Answer 10

Haemophilius influenza (1) Psuedomonus aureginosa is the most common causative pathogen for infective exacerbations

Question 11

Bronchiectasis : Acute exacerbation - clinical features

Answer 11

• Worsening of cough
• Increased volume of sputum or change in colour
• Fever, malaise
• Hypoxia if very severe

Question 12

Bronchiectasis : Acute exacerbation - management

Answer 12

1. Indication - clinical deterioration and new growth of pneudomonas auregenosa
   **First line*: 2 weeks of PO Ciprofloxacin
2. > 3 exacerbation in 1 years
   * Long term antibiotics
   - Inhaled or PO antibiotics

Question 13

Bronchiectasis : Acute exacerbation - Complications

Answer 13

1. Chronic hypoxia: due to poor lung function and gas exchange
2. Pulmonary hypertension
   * vasoconstriction of pulmonary arteries away from damaged tissue
   * Increased vasoconstriction, increases blood pressure in pulmonary vessels
   * RV has increased resistance to pump against leading to
3. Cor Pulmonale / Right heart failure