What are the pharmacological and non-pharmacological management strategies for acute exacerbations and long-term management of non-cystic fibrosis (non-CF) bronchiectasis in adults?

Management of Non-Cystic Fibrosis Bronchiectasis in Adults

Slide 1: Introduction to Non-CF Bronchiectasis

• Chronic lung condition characterized by permanent bronchial dilatation and inflammation 1
• Presents with chronic productive cough, recurrent infections, and impaired quality of life 1
• Prevalence increases with age (7 per 100,000 in ages 18-34 vs 812 per 100,000 in those ≥75 years) 1
• More common in women than men (180 vs 95 per 100,000) 1

Slide 2: Diagnosis and Initial Evaluation

• High-resolution CT (HRCT) scanning is the gold standard for diagnosis 2
• Initial diagnostic workup should include:
  • Complete blood count with differential 1
  • Immunoglobulin quantification (IgG, IgA, IgE, IgM) 1
  • Sputum cultures for bacteria, mycobacteria, and fungi 1
  • Pre- and post-bronchodilator spirometry 1
  • Testing for allergic bronchopulmonary aspergillosis 3

Slide 3: Management of Acute Exacerbations

• Exacerbations present with increased cough, sputum production, and worsened fatigue 1
• Obtain sputum culture prior to initiating antibiotics 3
• Treat exacerbations with 14 days of antibiotics 3
• Antibiotic selection should be guided by previous sputum cultures 2
• Consider IV antibiotics for severe exacerbations or treatment failures 3

Slide 4: Airway Clearance Techniques

• All patients with bronchiectasis should participate in airway clearance techniques 3
• Options include:
  • Active cycle of breathing techniques 3
  • Postural drainage 3
  • Manual techniques (percussion, vibration) 3
  • Mechanical devices (flutter, acapella) 3
• Sessions should last 10-30 minutes until two clear huffs or coughs are completed 3

Slide 5: Pulmonary Rehabilitation

• Patients with impaired exercise capacity should participate in pulmonary rehabilitation 3
• Benefits include:
  • Improved exercise capacity 3
  • Reduced cough symptoms 3
  • Enhanced quality of life 3
  • Potential reduction in exacerbation frequency 3
• Regular exercise should be maintained after formal rehabilitation 3

Slide 6: Mucoactive Treatments

• Consider for patients with difficulty expectorating sputum 2
• Do NOT use recombinant human DNase (rhDNase) in non-CF bronchiectasis 3
• Consider humidification with sterile water or normal saline to facilitate airway clearance 3
• Consider pre-treatment with bronchodilator before inhaled mucoactive treatments 3
• Trial for 6 months and continue if ongoing clinical benefit 3

Slide 7: Long-term Antibiotic Therapy - When to Consider

• Consider long-term antibiotics for patients with ≥3 exacerbations per year 3
• Reduces exacerbation frequency, time to first exacerbation, and improves quality of life 3
• Requires regular monitoring of sputum pathogens to track resistance patterns 2
• Antimicrobial stewardship is essential 3

Slide 8: Long-term Antibiotic Therapy - Pseudomonas aeruginosa Infection

• For chronic P. aeruginosa infection:
  • First line: Inhaled colistin 3
  • Second line: Inhaled gentamicin 3
  • Alternative: Macrolides (azithromycin, erythromycin) if inhaled antibiotics not tolerated 3
  • Consider combination therapy for frequent exacerbations despite inhaled antibiotics 3

Slide 9: Long-term Antibiotic Therapy - Non-Pseudomonas Infection

• For non-Pseudomonas infections:
  • First line: Macrolides (azithromycin, erythromycin) 3
  • Alternative: Targeted oral antibiotics based on susceptibility 3
  • Consider inhaled antibiotics if oral options contraindicated or ineffective 3
• Before starting macrolides:
  • Exclude active nontuberculous mycobacterial (NTM) infection 3
  • Use caution with significant hearing loss or balance issues 3

Slide 10: Anti-inflammatory Treatments

• Do not routinely offer inhaled corticosteroids unless comorbid asthma or COPD 3
• Do not offer long-term oral corticosteroids 3
• Do not offer PDE4 inhibitors, methylxanthines, or leukotriene receptor antagonists 3
• Do not offer CXCR2 antagonists, neutrophil elastase inhibitors, or statins 3

Slide 11: Special Considerations - NTM Management

• Carefully evaluate each respiratory NTM isolate in context of overall patient status 2
• Treatment should involve collaboration with experts in NTM and bronchiectasis 2
• Exclude NTM infection before starting macrolide therapy 3

Slide 12: Monitoring and Follow-up

• Regular monitoring of sputum pathogens before and after implementation of long-term antibiotics 2
• Monitor for drug toxicity, especially with macrolides and inhaled aminoglycosides 2
• Annual assessment by respiratory physiotherapist to optimize airway clearance regimen 3
• Review airway clearance technique if condition deteriorates 3

Slide 13: Stepwise Management Approach

• Step 1: Optimize airway clearance techniques and patient education 3
• Step 2: Reassess physiotherapy and consider mucoactive treatment if ≥3 exacerbations/year 3
• Step 3: Add long-term antibiotics based on pathogen if still ≥3 exacerbations/year:
  • P. aeruginosa: Inhaled anti-pseudomonal antibiotic or macrolide 3
  • Other pathogens: Macrolides or targeted antibiotics 3
  • No pathogen: Macrolides 3
• Step 4: Consider combination therapy or specialist referral if continued exacerbations 3

Slide 14: Guidelines Referenced

• European Respiratory Society Guidelines (2017) 3
• British Thoracic Society Guidelines (2019) 3
• American College of Chest Physicians recommendations (via Praxis Medical Insights) 2