Bronchiectasis

Question 1

What is Bronchiectasis?

Answer 1

chronic respiratory disease characterised by permanent bronchial dilation, due to irreversible damage to the bronchial wall.

Question 2

How does it occur?

Answer 2

An initial insult to the bronchi (e.g. infection) results in immune cells being recruited to the bronchi. These immune cells secrete cytokines and proteases, leading to inflammation in the bronchi. This inflammation damages the muscle and elastin found in the bronchial walls, leading to bronchial dilation.Dilated bronchi are predisposed to persistent microbial colonisation, as mucus traps in the dilated bronchi

Question 3

causes?

Answer 3

Recurrent childhood lower respiratory tract infections (e.g. influenza, pertussis and measles)
Pulmonary tuberculosis
Allergic bronchopulmonary aspergillosis (ABPA)
Chronic obstructive pulmonary disease
Asthma
Cystic fibrosis
Primary ciliary dyskinesia
Alpha-1 antitrypsin deficiency (also causes emphysema)
Rheumatoid arthritis
Systemic lupus erythematosus (SLE)
Sarcoidosis

Question 4

Risk factors?

Answer 4

Age (>70 years)
Female gender
Smoking history

Question 5

Symptoms?

Answer 5

Daily cough, producing copious amounts of mucopurulent sputum (haemoptysis is present in 50% of patients)
Exertional dyspnoea, which may progress to resting dyspnoea
Fatigue
Rhinosinusitis symptoms (such as nasal discharge, nasal obstruction and facial pressure): due to underlying mucociliary impairment
History of childhood lower respiratory tract infections: you may forget to ask this if the patient is elderly
Family history: ask about congenital conditions (such as cystic fibrosis) and autoimmune conditions (such as rheumatoid arthritis)
Smoking history: quantify in pack-years (1 pack-year = smoking 20 cigarettes a day for a year)
Clinical examinati

Question 6

Findings on examination?

Answer 6

Finger clubbing: due to increased secretion of growth factors, leading to increased growth of extracellular matrix in the nails
Course crepitations, present in expiration and inspiration: caused by sudden opening and closing of the airways
Rhonchi (low-pitched noises, which sound like snoring): caused by the movement of secretions in the large airways
High-pitched inspiratory squeaks and pops: unclear aetiology

Question 7

Investigation?

Answer 7

Full blood count: may show elevated white blood cell count, including neutrophilia
CRP: may be elevated during acute exacerbations
Autoimmune screen (if suspecting an autoimmune condition): includes anti-CCP, ANA and ANCA
Specific IgE to Aspergillus fumigatus: if suspecting ABPA
Genetic testing (done in specialist units): to diagnose congenital disorders, such as cystic fibrosis and primary ciliary dyskinesia
Chest X-ray: required to exclude other pathologies.
High-resolution CT chest
Bronchoscopy- localised bronchiectasis

Question 8

Management?

Answer 8

• pulmonary rehab
• smoking cessation
  -flu vaccine
  -Long-term antibiotics (e.g. azithromycin three times a week)
  -Bronchodilators: offer a long-acting bronchodilator (e.g. formoterol)
• CFTR modulator therapies
  -lung resection/ transplant