What is the recommended approach to investigate and treat bronchiectasis?

Investigation and Treatment of Bronchiectasis

Bronchiectasis requires a comprehensive diagnostic workup followed by targeted treatment focusing on airway clearance, infection control, and management of underlying causes to reduce exacerbations and improve quality of life. 1, 2

Diagnostic Investigation

Initial Imaging

• Start with baseline chest X-ray in patients with suspected bronchiectasis 1
• Confirm diagnosis with thin-section CT scan when clinically suspected 1
• Perform imaging during clinically stable disease for optimal diagnostic accuracy 1

CT Diagnostic Criteria

Bronchiectasis is defined by bronchial dilatation with one or more of:

• Bronchoarterial ratio >1 (internal airway lumen vs adjacent pulmonary artery)
• Lack of airway tapering
• Airway visibility within 1cm of pleural surface 1

When to Suspect and Investigate

Consider investigation for bronchiectasis in:

• Patients with persistent production of mucopurulent/purulent sputum 1
• Patients with COPD having ≥2 exacerbations annually and previous P. aeruginosa culture 1
• Rheumatoid arthritis patients with chronic productive cough/recurrent infections 1
• Inflammatory bowel disease patients with chronic productive cough 1
• Patients with cough persisting >8 weeks, especially with sputum production 1
• Patients with asthma with severe or poorly-controlled disease 1

Essential Diagnostic Workup

1. Minimum testing bundle:

   • Complete blood count with differential
   • Immunoglobulin quantification (IgG, IgA, IgE, IgM)
   • Testing for allergic bronchopulmonary aspergillosis
   • Sputum cultures for bacteria, mycobacteria, and fungi 3

2. Additional targeted investigations:

   • Consider bronchoscopy for patients with localized disease to rule out endobronchial lesions/foreign bodies 1
   • Bronchial aspiration/wash for patients who don't expectorate (especially helpful for NTM diagnosis) 1
   • Consider HIV testing based on risk factors 1
   • Investigations for reflux/aspiration only in symptomatic patients 1

Treatment Approach

Airway Clearance and Hydration

• Implement airway clearance techniques 1-2 times daily 2
• Techniques should be taught by respiratory physiotherapist:
  • Active cycle of breathing
  • Autogenic drainage
  • Postural drainage
  • Device-assisted methods 2
• Ensure adequate hydration to thin secretions 2
• Consider humidification with sterile water/normal saline 2
• Consider mucoactive treatments for difficult expectoration (avoid DNase) 2

Antibiotic Therapy

1. For acute exacerbations:

   • Select antibiotics based on previous sputum cultures, exacerbation severity, and clinical response 2
   • Obtain sputum cultures before starting antibiotics when possible 2
   • Standard treatment duration is 14 days 2
   • Consider IV antibiotics for severe symptoms, treatment failures, or resistant P. aeruginosa 2

2. For P. aeruginosa eradication:

   • First-line: ciprofloxacin 500-750mg twice daily for 2 weeks 2

3. For frequent exacerbations (≥3 per year):

   • Consider long-term macrolides (e.g., azithromycin) 2, 3
   • Consider inhaled antibiotics (colistin, gentamicin) for P. aeruginosa infection 2, 3

Anti-inflammatory Treatment

• Avoid routine inhaled corticosteroids without specific indications (ABPA, asthma, COPD) 2
• Avoid long-term oral corticosteroids without specific indications 2
• For ABPA: initial prednisolone 0.5mg/kg/day for 2 weeks, then wean 2
• Consider itraconazole as steroid-sparing agent for steroid-dependent ABPA 2

Pulmonary Rehabilitation and Vaccination

• Recommend regular exercise for patients with impaired exercise capacity 2
• Offer pulmonary rehabilitation to improve exercise capacity and reduce exacerbations 2
• Provide annual influenza vaccination to all patients 2
• Offer pneumococcal vaccination to all patients 2

Advanced Management

• Consider long-term oxygen therapy for patients with respiratory failure 2
• Consider non-invasive ventilation with humidification for hypercapnic respiratory failure 2
• Consider lung resection for localized disease uncontrolled by medical treatment 2
• Consider lung transplant referral for patients ≤65 years with FEV1 <30% and clinical instability 2

Monitoring and Follow-up

• Assess patients annually (more frequently in severe disease) 2
• Perform pulse oximetry to screen for respiratory failure 2
• Send sputum for culture before and after eradication antibiotics 2
• Monitor for antibiotic resistance with repeat sensitivity testing 2
• Record weight and BMI at each clinic appointment 2
• Review by respiratory physiotherapist within 3 months of initial assessment 2

Common Pitfalls to Avoid

1. Failing to investigate for underlying causes that may require specific treatment
2. Using recombinant human DNase (effective in CF but harmful in non-CF bronchiectasis)
3. Prescribing inhaled corticosteroids without appropriate indications
4. Inadequate duration of antibiotic therapy for exacerbations
5. Not implementing regular airway clearance techniques
6. Missing treatable conditions like immunodeficiency or ABPA