Background Information

Definitions

Bronchiectasis: chronic, progressive lung disease characterised by irreversible bronchial dilation (focal or diffuse).

Exacerbation of bronchiectasis: persistent ≥48h worsening with ≥3 of: increased cough, sputum (volume/consistency), sputum purulence, breathlessness, fatigue/malaise, or haemoptysis, requiring a clinician-directed treatment change.^[Ref]

Aetiology

Bronchiectasis

The following mechanisms contribute to the pathogenesis of bronchiectasis: ^[Ref]

• Respiratory infections or inflammation 
• Airway obstruction 

Specific causes

• Idiopathic (~40% of cases)
• Chronic or recurrent respiratory infections
• Airway obstruction 
  • Disorders of secretion / mucociliary clearance
    • Cystic fibrosis
    •  Primary ciliary dyskinesia (e.g., Kartagener syndrome)
  • Other
    • COPD
    • Aspiration 
    • Tumors 
• Immunodeficiency (e.g., HIV, congenital immunodeficiencies) 
• Allergic bronchopulmonary aspergillosis (ABPA)
• Systemic inflammatory diseases (e.g. rheumatoid arthritis, IBD) 
• Rare syndromes / congenital defects

Pathogens 

• Several pathogens colonise the airways and are implicated in the development / progression of bronchiectasis 
• Most common:
  • Haemophilus influenzae (20-40%) 
  • Pseudomonas aureginosa (10-30%)

​​​​​​Exacerbations

Main cause → pathogen airway colonisation or acute infection ^[Ref]

• Bacteria 
  • Haemophilus influenzae
  • Pseudomonas
  • Staphylococcus aureus
• Fungi (esp. Aspergillus spp.)
• Viruses (e.g., influenza, rhinovirus, coronaviruses)

Clinical Features

Bronchiectasis

Symptoms

• Common 
  • Chronic productive cough with expectoration of large volumes of purulent sputum 
  • Dyspnoea 
  • Rhinosinusitis 
• Additional 
  • Haemoptysis (minor → massive)
  • Nonspecific symptoms → Fatigue, Weight loss, Fever 
  • Non-pleuritic chest pain 

O/E

• Signs
  • Nail clubbing (uncommon)
• Auscultation
  • Coarse crackles (esp lower zones)
  • Wheezing 

Exacerbation of bronchiectasis 

Suspect if:

• There is a worsening of common symptoms from baseline AND/OR
• Development of new symptoms such as fever, pleuritic chest pain or haemoptysis

Complications

Exacerbations 

• Very common & accelerates disease progression 

Pulmonary 

• Haemoptysis → blood in sputum is common; may be massive (>250ml) and life-threatening
• Respiratory failure → from progressive lung damage 
• Cor pulmonale 

Prognosis

• Mild disease → Normal life expectancy possible.
• Worse prognosis with:
  • Extensive disease
  • Frequent/severe exacerbations 
  • Breathlessness (strong mortality predictor) 
  • Chronic Pseudomonas colonisation
  • Comorbidities, smoking 

Guidelines

Investigation and Diagnosis

Diagnostic Tests

• 1st line: chest X-ray 
• Confirmatory test: HRCT

 

Imaging modality	Interpretation
Chest X-ray	Note that chest X-ray can often be normal or non-diagnostic Possible findings: Tram-track opacities (parallel lines indicative of dilated bronchi) Ring shadows (indicative of dilated terminal bronchi) Coarsened lung markings Increased bronchovascular markings
HRCT	Signet ring sign – diameter of bronchus > adjacent pulmonary artery Bronchial dilatation Lack of bronchial tapering Visualisation of bronchi within 1cm of pleural surface (normally airways are not seen so peripherally)

Investigations for Causes

• Test for cystic fibrosis
• Test for primary ciliary dyskinesia (if there is a history of neonatal distress / symptoms from childhood / recurrent otitis media / rhinosinusitis / infertility)
• Sputum culture
• Antibody testing
  • Screen for antibody deficiency
  • Test serum total IgE and specific IgE or skin prick test to Aspergillus (to exclude allergic bronchopulmonary aspergillosis)
  • Test specific antibody levels against capsular polysaccharides of Streptococcus pneumoniae

Consider other testing:

• Rheumatology serology screening (RF, Anti-CCP, ANA, ANCA)
• Test for alpha 1 antitrypsin deficiency
• HIV-1 serology
• Serum protein electrophoresis (in those with raised immunoglobulins)
• Bronchoscopy (to rule out endobronchial lesion or foreign body aspiration)

Management

Long-Term Management

All patients:

• Airway clearance technique (taught by physiotherapist)
• Smoking cessation
• Immunisation against Streptococcus pneumoniae, annual influenzae, COVID-19

Consider:

• Humidification with normal saline / sterile water to facilitate airway clearance
• Long-acting bronchodilator in those with significant breathlessness

 

Antibiotic Prophylaxis

Do not routinely offer antibiotic prophylaxis.

Antibiotic prophylaxis may be offered if there are ≥3 exacerbations per year (only offered by specialist)

• 1st line for most patient: azithromycin / erythromycin
• If there is concurrent Pseudomonas aeruginosa infection: inhaled colistin
• Offer antibiotic prophylaxis for at least 6 months

 

Surgical Management

Consider lung resection in localised disease and not controlled by medical therapy.

Consider referral for lung transplantation if:

• ≤65 y/o, and
• FEV₁ <30%, and
• Significant clinical instability or rapid progressive respiratory deterioration despite optimal medical therapy

Infective Exacerbation Management

Obtain a sputum sample (for culture and susceptibility testing), then offer an antibiotic

• If previous microbiology cultures are available, use them to guide antibiotic choice

 

No Previous Microbiology Cultures Available

Standard empirical antibiotic choice (for 7-14 days):

• 1st line: amoxicillin
• 2nd line: clarithromycin / doxycycline 

If there is high risk of treatment failure:

• 1st line: co-amoxiclav
• 2nd line: levofloxacin 

Choice for IV antibiotic therapy (if unable to take oral antibiotic or severely unwell):

• 1st line: co-amoxiclav / tazocin (piperacillin with tazobactam)
• 2nd line: levofloxacin

Previous Microbiology Cultures Available

1st line antibiotics for common organisms from NICE CKS:
 

Organism	1st line antibiotic
Streptococcus pneumoniae	Amoxicillin
Haemophilus influenzae	Amoxicillin (if beta-lactamase -ve) Co-amoxiclav (if beta-lactamase +ve)
Moraxella catarrhalis	Co-amoxiclav 625 mg three times daily
Staphylococcus aureus	Flucloxacillin 500 mg four times daily
MRSA	Rifampicin + doxycycline (if <50 kg) Rifampicin + trimethoprim (if >50 kg)
Coliforms (e.g. Klebsiella or Enterobacter)	Ciprofloxacin
Pseudomonas aeruginosa	Ciprofloxacin

References

Original Guideline