Bronchiectasis

Background/Epidemiology

• Bronchiectasis is a permanent dilation and thickening of the airways associated with chronic cough, sputum production, bacterial colonisation and recurrent infection
  • Chronic suppurative airway infection with sputum production, progressive scarring and lung damage occure
• It can be focal or diffuse
• It is estimated that 1 in 1000 adults in the UK have bronchiectasis

Aetiology

• Many possible causes
  • Congenital abnormalities
    • e.g. cystic fibrosis, ciliary dysfunction syndromes (e.g. Primary ciliary dyskinesia (immotile cilia syndrome); Kartagener’s syndrome; Young’s syndrome), and primary hypogammaglobulinaemia (immunodeficiency);
  • Acquired causes
    • Children
      • Pneumonia
      • Primary TB
      • Inhaled foreign body
    • Adults
      • Post infective
        • Suppurative pneumonia
        • Pulmonary TB
        • Measles
        • Pertussis
      • Allergic bronchopulmonary aspergillosis complicating asthma
      • Bronchial tumours/obstruction
      • Some connective tissue disorders e.g. rheumatoid arthritis, Sjogren’s
      • Asthma can be a cause (but only considered if no other cause found)
      • GORD/Reflux aspiration (chronic)

Pathophysiology

• Whatever the cause, chronic inflammation of the airways occurs
• Granulation tissue production and eventually fibrosis occurs, which can lead to damage to the normal lung architecture
• This predisposes to further infection/inflammation and the cycle continues

Clinical Presentation

• Bronchiectasis should be considered in a child who presents with
  • Chronic moist/productive cough, especially between viral colds or with positive bacterial cultures
    • Wheeze and exertional SOB may also be features
  • Asthma that does not respond to treatment
  • An episode of severe pneumonia (or recurrent/multiple episodes of pneumonia) with incomplete resolution of symptoms
  • Unexplained haemoptysis
  • Respiratory symptoms with any clinical features of CF, PCD or immunodeficiency
    • e.g. finger clubbing, cyanosis, hyperinflation
  • There may also be a history of failure to thrive and signs of malnutrition
• In adults
  • persistent, productive cough
    • young patient or a long history of symptoms
    • absence of smoking history
    • daily expectoration of large volumes of very purulent sputum
    • haemoptysis
    • Pseudomonas aeruginosa in the sputum
  • Other symptoms include shortness of breath and chest pain (often non-pleuritic)
  • who are thought to have COPD but who do not smoke, or have frequent/prolonged/difficult to manage exacerbations
    • there may also be a history of a severe lower respiratory tract infection (remember TB) during childhood
• Signs include
  • Coarse crackles during early inspiration that are most common in the lower lung fields
  • Wheeze
  • Finger clubbing (relatively rare unless associated with CF)

Investigations

• CXR should be arranged in the first instance to exclude other causes (see cough)
• High-resolution CT can be diagnostic (shows bronchial wall dilation)
• Lung function tests
• Other investigations are usually required to identify an underlying cause, e.g.
  • Sweat test for CF (all patients <40 years old and adults >40 with features consistent with CF)
  • Immunodeficiency screen (serum IgG, IgA, IgM and electrophoresis) (all patients)
  • Alpha-1 antitrypsin level, serum IgE and aspegillus IgE for patients with risk factors for immunological disease
  • Bronchoscopy- particularly in focal disease, obstructive causes e.g. foreign body or neoplastic causes

Management

• Chronic management is largely supportive
  • Smoking cessation
  • Annual follow-up
    • Sputum culture at each follow-up (and between if increase in purulence/volume)
  • Education- known how to recognise exacerbations
    • Delayed antibiotic prescribing may be appropriate
  • Offer immunisations e.g. flu
• Consider referral in
  • All children
  • Chronic colonisation with Pseudomonas aeruginosa, opportunist mycobacteria or MRSA
  • Deteriorating symptoms/significant morbidity (increasing volume/purulence of sputum, increasing SOB etc)
  • 3 or more exacerbations/year (which may require long-term antibiotics
• Physiotherapy education for mucus/lung clearance is extremely important (inspiratory muscle training and postural drainage)
• Infective exacerbations (characterised by worsening symptoms) should be treated with empirical antibiotics (these may be based on the individual, based on recommendations for patients with bronchiectasis or based on recommendations for the general population- if uncertain, consult ID/Microbiology) AFTER a sputum sample has been sent for culture
  • Choice of antibiotic should ideally be in line with local guidance
    • (Here is local guidance for CF; Here are some recommendations for exacerbations of bronchiectasis from NICE)

Complications

• Amyloidosis can occur as a result of the chronic inflammation