Bronchiectasis

Overview

Bronchiectasis is a persistent or progressive chronic lung condition characterised by the permanent dilation of the bronchi due to permanent damage to the bronchial wall elastic and muscular tissue. This leads to copious sputum production, bacterial colonisation, and recurrent infections.

Bronchiectasis is caused by chronic inflammation of the airways and may develop after lung infections in childhood and in association with other conditions such as immunodeficiency or cystic fibrosis.

Epidemiology

• Prevalence is higher in women than in men
• Prevalence is higher >60 years

Causes

• Previous infection, particularly in childhood – most common
  • Examples are tuberculosis, severe bacterial pneumonia, measles, pertussis and influenza
• Immunodeficiency including HIV
• Connective tissue disease (e.g. systemic lupus erythematosus)
• Congenital causes:
  • Cystic fibrosis
  • Kartagener’s syndrome (primary ciliary dyskinesia)
  • Alpha-1-antitrypsin deficiency
• Bronchial obstruction (e.g. tumours, lymphadenopathy, or inhaled foreign bodies)

Presentation

Patients typically have a chronic cough with copious amounts of purulent sputum. Features are:

• Cough – associated with large amounts of purulent sputum
  • There may be mild haemoptysis (small flecks of blood)
• Crackles on auscultation:
  • Low-pitched crackles (rhonchi) are heard which are often cleared with coughing
• Shortness of breath
• Recurrent episodes of fever
• Weight loss
• Finger clubbing – this is rare in bronchiectasis

Features of an acute exacerbation:

• Recurrence of fever
• Increased sputum production
• Change in colour of sputum

Differential Diagnoses

Chronic obstructive pulmonary disease (COPD)

• Diminished breath sounds are heard in COPD but not in bronchiectasis
• Patient usually has a history of smoking
• Rhonchi are present in bronchiectasis which often clears on coughing

Asthma

• Symptoms are usually diurnal (vary throughout the day, usually worse at night and first thing in the morning)
• The cough is usually dry
• There is usually a history of associated atopy e.g. allergic rhinitis, eczema, etc. 

Heart failure

• The patient may have a history of ischaemic heart disease or a myocardial infarction
• There may be oedema, pulmonary crackles, or an elevated jugular venous pressure (JVP) on examination

Investigations

All patients

• Chest x-ray:
  • Should be done in all patients as a baseline
  • May show:
    • Ring or tubular opacities
    • Tramlines 
• High-resolution CT chest:
  • Gold-standard for diagnosis
  • May show:
    • Thickened and dilated airways
    • A ‘signet ring’ sign
• Sputum culture and sensitivity:
  • Commonly isolated organisms are:
    • Haemophilus influenzae – most common
    • Pseudomonas aeruginosa

Other investigations

Investigations to determine the underlying cause may include:

• Cystic fibrosis testing
• Screening for antibody deficiency (IgG, IgA, IgM)
• Serum total IgE and specific IgE – to exclude allergic bronchopulmonary aspergillosis
• Antibody levels against Streptococcus pneumoniae
• Testing for primary ciliary dyskinesia
• Testing for connective tissue disorders:
  • Rheumatoid factor (RF)
  • Anti-cyclic citrullinated peptide (anti-CCP) antibodies
  • Anti-nuclear antibodies (ANA)
  • Anti-neutrophil cytoplasmic antibodies (ANCA)
• HIV testing
• Testing for alpha-1-antitrypsin deficiency
• Bronchoscopy – if there is localised disease to rule out lesions within the bronchi (e.g. tumours) or foreign body aspiration

Management

All patients

The damage to the lungs cannot be reversed. Management involves preventing or slowing down further deterioration.

• Regular follow-up in secondary care
• Lifestyle changes and measures (see patient advice)
• Influenza and pneumococcal vaccination
• Postural drainage and airway clearance physiotherapy techniques
• Antibiotics in acute exacerbations
• Consider surgery if there are areas of localised disease

Monitoring

• Patients are reviewed at least annually to assess symptom severity
• Patients with multiple exacerbations may be considered for long-term antibiotic prophylaxis
• Patients should have sputum cultures done at follow-up

Patient Advice

• Maintain a healthy diet and adequate physical exercise
• Stop smoking and avoid passive smoking – they should be offered help for this
• Daily airway clearance techniques should be carried out – these are taught by physiotherapists
• Patients should seek help if signs of an acute exacerbation arise (e.g. fever, change in sputum colour or volume, new-onset or worsening shortness of breath) 

Complications

• Infective exacerbations
• Chronic bacterial colonisation
• Haemoptysis
• Pneumothorax
• Respiratory failure
• Cor pulmonale
• Coronary heart disease and ischaemic stroke
• Reduced quality of life – the impact is the same as severe COPD

Prognosis

• The prognosis varies significantly. It is worse in people:
  • With severe disease
  • Who have frequent exacerbations
  • Who have breathlessness
  • Who have an underlying disease
  • With comorbidities
  • Who smoke