Bronchiectasis

Question 1

What is bronchiectasis?

Answer 1

Bronchiectasis is permanent dilation of the bronchi and bronchioles following direct damage to the airway walls, most commonly by focal infections. This leads to mucus plugging and further recurrent infections.

The dilatation causes obstructive lung disease – it is therefore categorised alongside asthma and COPD.

Question 2

What are the 2 ways you can classify bronchiectasis?

Answer 2

Classified as either CF bronchiectasis or non-CF bronchiectasis

Question 3

Describe the aeitology of bronchiectasis

Answer 3

● Chronic lung inflammation leads to fibrosis and permanent dilation of the bronchi due to destruction of the elastic and muscular components of the bronchial wall
● This leads to pooling of mucus, which predisposes to further cycles of infection, damage and fibrosis of bronchial walls

Question 4

What are some causes of bronchiectasis?

Answer 4

Use the mnenmonic II CRAM:
1. Immune OVER-response – allergic bronchopulmonary aspergillosis (ABPA)
2. Immune UNDER-response – HIV, inherited immunodeficiencies, bone marrow suppression
3. Congenital – cystic fibrosis, Kartagener syndrome, primary ciliary dyskinesia, Young syndrome, yellow nail syndrome
- almost all patients with cystic fibrosis will ultimately develop severe bronchiectasis
4. Childhood infection – tuberculosis, measles, pertussis, pneumonia
5. Respiratory disease – COPD, pulmonary fibrosis
- pulmonary fibrosis causes a ‘traction bronchiectasis’
as the interstitium fibroses, it contracts, thereby pulling the bronchiolar walls apart
6. Aspiration pneumonia
7. Mechanical obstruction – foreign body, tumour, lymph node, external compression

Question 5

What is cystic fibrosis?

Answer 5

• Autosomal recessive disorder causing increased viscosity of secretions.
• Due to a defect in the the cystic fibrosis transmembrane conductance regulator gene (CFTR), which codes a chloride channel.
• May cause recurrent chest infections, failure to thrive (delayed puberty), malabsorption (steatorrhoea), short stature, male infertility.
• Diagnosed via sweat test (will have abnormally high sweat chloride).
• Typically screened at birth via heel prick test.
• Mx involves chest physiotherapy.

Question 6

What is Primary Ciliary Dyskinesia (Kartagener’s Syndrome)?

Answer 6

• Rare, autosomal recessive genetic ciliary disorder comprising the triad of: situs inversus (a rare genetic condition in which the organs in your chest and abdomen are positioned on the wrong side, inlcuding heart and testes), chronic sinusitis, and bronchiectasis.
• Can also cause male infertility.

Question 7

What is Allergic Bronchopulmonary Aspergillosis?

Answer 7

• Bronchiectasis + bronchoconstriction (wheeze, cough, dyspnoea - patient may have previous label of asthma).
• Will also have eosinophilia and raised IgE.
• Tx with oral prednisolone.
• Hypersensitivity response to the fungus aspergillus.

Question 8

Summarise the epidemiology of bronchiectasis

Answer 8

● Most often arises initially in CHILDHOOD
● Incidence has decreased with the use of antibiotics
● 1/1000 per year
● Tall, thin, white females aged 60 or over are at greater risk of pulmonary non-tuberculous mycobacteria related bronchiectasis

Question 9

What are the presenting symptoms of bronchiectasis?

Answer 9

● KEY: Persistent cough with copious purulent sputum (large amount of off-white, yellow or green, and opaque sputum. It indicates the presence of large numbers of white blood cells) – may be worsened by lying flat
● Intermittent haemoptysis
● Breathlessness
● Chest pain
● Malaise
● Fever – recurrent episodes
● Weight loss
● Symptoms usually begin after an acute respiratory illness
● All symptoms worsen during acute exacerbations

Question 10

What signs of bronchiectasis can be found on physical examination?

Answer 10

1. Clubbing
2. Coarse inspiratory crackles (usually at lung bases)
   o These shift with coughing
3. Wheeze – high-pitched inspiratory squeaks

Question 11

What investigations are used to diagnose/ manage bronchiectasis?

Answer 11

1. CXR (initial test) → shows obscured hemidiaphragm, thin-walled ring shadows with or without fluid levels, tram lines (parallel line shadows), tubular or ovoid opacities
2. High-Resolution Chest CT (confirmatory test) → gold standard. Thickened, dilated airways with or without air fluid levels; varicose constrictions along airways; cysts and/or tree-in-bud pattern. Signet ring sign. Tram-track sign.
3. Spirometry → obstructive pattern (<0.7)
4. Perform other tests (eg. FBC, sputum culture, RF) to look for underlying aetiology
   - Sputum MCS ⇒ may find haemophilus influenzae (most common) or pseudomonas aeruginosa (most common in CF patients)
   - Cystic Fibrosis Sweat Test (abnormally high chloride)

Question 12

How is bronchiectasis managed?

Answer 12

1. Exercise and Improved Nutrition
2. Airway Clearance Therapy (Chest Physiotherapy) → maintenance of oral hydration, postural drainage (x2 per day), percussion, vibration, and the use of oscillatory devices
3. Inhaled Bronchodilator (Salbutamol)
4. Mucoactive Agent → nebulised hypertonic saline
5. Antibiotics → amoxicillin, vancomycin etc.
6. Immunisations
7. Indications for Surgery (Lobectomy) ⇒ uncontrollable haemoptysis or localised disease

Question 13

What complications may arise following bronchiectasis?

Answer 13

● Life-threatening haemoptysis
● Pneumonia
● Pneumothorax
● Persistent infections
● Empyema
● Respiratory failure
● Cor pulmonale
● Multi-organ abscesses
● Amyloidosis

Question 14

Describe the prognosis of bronchiectasis?

Answer 14

● Most patients continue to have symptoms after 10 years