What is the management of cystic bronchiectasis?

Management of Cystic Bronchiectasis

All adults with cystic bronchiectasis and chronic productive cough should be taught airway clearance techniques by a trained respiratory physiotherapist, performing sessions for 10-30 minutes once or twice daily, and those with impaired exercise capacity must participate in pulmonary rehabilitation programs. 1

Diagnostic Workup

When cystic bronchiectasis is newly diagnosed, perform the following minimum bundle of aetiological tests 1:

• Differential blood count to identify immunological abnormalities
• Serum immunoglobulins (total IgG, IgA, IgM) to detect immunodeficiency states
• Testing for allergic bronchopulmonary aspergillosis (ABPA) as a treatable cause
• Sputum culture and sensitivity for bacterial monitoring, with mycobacterial culture in selected cases where non-tuberculous mycobacteria are suspected 1

Non-Pharmacological Management (Foundation of Treatment)

Airway Clearance Techniques (ACTs)

ACTs are mandatory for all patients with chronic productive cough or difficulty expectorating sputum 1, 2:

• Sessions should last 10-30 minutes, performed once or twice daily 2, 3
• Techniques include active cycle of breathing, autogenic drainage, or devices such as Flutter or Acapella that modify expiratory flow and produce chest wall oscillations 1
• ACTs increase sputum volume, reduce cough impact on quality of life, and may reduce peripheral airways obstruction and inflammatory cells in sputum 1, 4
• Despite weak evidence quality due to small studies, most demonstrate significant increases in sputum volume 1

Pulmonary Rehabilitation

Strongly recommended for all patients with impaired exercise capacity 1:

• Consists of 6-8 weeks of supervised exercise training with review of airway clearance techniques 1
• Improves exercise capacity immediately after the program, with benefits maintained for 3-6 months 1
• Reduces exacerbation frequency (median 1 vs 2 exacerbations; p=0.012) and prolongs time to first exacerbation (8 vs 6 months; p=0.047) 1
• Enhances quality of life and reduces cough symptoms 1

Pharmacological Management

Bronchodilators

• Use bronchodilators before physiotherapy sessions, inhaled mucoactive drugs, and inhaled antibiotics to increase tolerability and optimize pulmonary deposition 1
• Offer long-acting bronchodilators for patients with significant breathlessness on an individual basis 1
• Do not routinely offer long-acting bronchodilators to all patients with bronchiectasis 1
• Continue bronchodilators in patients with comorbid asthma or COPD regardless of bronchiectasis diagnosis 1

Mucoactive Treatments

• Offer long-term mucoactive treatment (≥3 months) for patients with difficulty expectorating sputum and poor quality of life where standard airway clearance techniques have failed 1
• Do NOT offer recombinant human DNase (dornase alfa) to adults with non-CF bronchiectasis—this is a strong recommendation based on moderate quality evidence 1

Management of Acute Exacerbations

Treat all acute exacerbations with 14 days of antibiotics 1, 2, 3:

• Select antibiotics based on previous sputum culture results 1, 2, 3
• Obtain sputum cultures before starting antibiotics whenever possible 3
• Shorter or longer courses may be appropriate depending on exacerbation severity, patient response, or specific microbiology 1

Common pathogens and first-line treatments 2, 3:

• Streptococcus pneumoniae: Amoxicillin 500mg three times daily for 14 days
• Haemophilus influenzae (beta-lactamase negative): Amoxicillin 500mg three times daily for 14 days
• Pseudomonas aeruginosa: Ciprofloxacin 500-750mg twice daily for 14 days

Long-Term Antibiotic Therapy

Consider long-term antibiotics only for patients with ≥3 exacerbations per year 1, 3:

For Chronic Pseudomonas aeruginosa Infection

• First-line: Long-term inhaled antibiotics (conditional recommendation, moderate quality evidence) 1
• Alternative: Macrolides (azithromycin or erythromycin) if inhaled antibiotics are contraindicated, not tolerated, or not feasible 1
• Consider adding macrolides to inhaled antibiotics for patients with high exacerbation frequency despite inhaled antibiotic therapy 1
• P. aeruginosa infection is associated with three-fold increase in mortality risk and almost seven-fold increase in hospitalization risk 3

For New Isolation of Pseudomonas aeruginosa

Offer eradication antibiotic treatment for new P. aeruginosa isolation (conditional recommendation, very low quality evidence) 1

For Patients Without Pseudomonas aeruginosa

• Long-term macrolides (azithromycin or erythromycin) are suggested for patients with ≥3 exacerbations per year 1
• Do NOT offer eradication treatment for new isolation of pathogens other than P. aeruginosa 1

Anti-Inflammatory Treatments

Do NOT offer inhaled corticosteroids to adults with bronchiectasis unless comorbid asthma or COPD is present (conditional recommendation, low quality evidence) 1, 3

Do NOT offer statins for treatment of bronchiectasis (strong recommendation, low quality evidence) 1

Surgical Management

Surgery is NOT recommended except for highly selected patients 1:

• Consider surgery only for patients with localized disease and high exacerbation frequency despite optimization of all other management aspects 1
• Pooled mortality from surgery is 1.4% (95% CI 0.8%-2.5%) 1
• Post-operative morbidity is 16.2% (95% CI 12.5%-19.8%), though some is relatively minor (air leak, atelectasis, wound infection) 1
• Complete symptom alleviation occurs in 71.5% (95% CI 68-74.9%) and symptom reduction in 20.2% (95% CI 17.3-23.1%) 1
• Unfavorable prognostic factors include extent of residual bronchiectasis and P. aeruginosa infection 1

Monitoring and Follow-Up

• Annual assessments minimum, with more frequent monitoring in severe disease 2
• Perform pulse oximetry to screen for respiratory failure 2
• Monitor sputum culture and sensitivity regularly 2
• For patients with constrictive bronchiolitis, closely monitor for disease progression and perform aggressive airway clearance to prevent mucus stasis 2

Critical Pitfalls to Avoid

• Do NOT extrapolate treatments from cystic fibrosis bronchiectasis—treatment responses differ significantly 3
• Do NOT use recombinant human DNase in non-CF bronchiectasis—this can be harmful 1, 3
• Do NOT routinely prescribe inhaled corticosteroids without comorbid asthma or COPD 1, 3
• Do NOT offer long-term antibiotics to patients with fewer than 3 exacerbations per year 1
• Ensure airway clearance techniques and general bronchiectasis management are optimized before considering long-term antibiotic therapy 1