What is the initial management for a patient with cystic bronchiectasis?

Initial Management of Cystic Bronchiectasis

All patients with cystic bronchiectasis should begin airway clearance techniques taught by a trained respiratory physiotherapist, performed once or twice daily, combined with regular physical exercise or pulmonary rehabilitation for those with impaired exercise capacity. 1

Diagnostic Workup and Baseline Assessment

Before initiating treatment, complete the following essential investigations:

• Confirm diagnosis with thin-section CT chest showing permanent bronchial dilatation 1
• Sputum cultures for routine bacteria, mycobacteria, and fungi while clinically stable 1
• Blood work: Complete blood count, serum IgG, IgA, IgE, and IgM to exclude immunodeficiency 1
• Spirometry: Pre- and post-bronchodilator testing 2
• Screen for underlying causes: Test for cystic fibrosis (if clinical features suggest), primary ciliary dyskinesia (nasal nitric oxide), ABPA (total IgE and Aspergillus-specific IgE), and specific antibody deficiency 1

Core Non-Pharmacological Management

Airway Clearance Techniques (ACTs)

Patients with chronic productive cough or difficulty expectorating should be taught an airway clearance technique by a trained respiratory physiotherapist. 1

• Frequency: Once or twice daily, minimum 10 minutes up to maximum 30 minutes 1
• Techniques to consider: Active cycle of breathing, autogenic drainage, positive expiratory pressure devices, oscillating PEP devices (Flutter, Acapella), high-frequency chest wall oscillation 1
• Primary benefit: Increased sputum volume and reduced impact of cough on quality of life 1
• Review timing: Within 3 months of initial assessment, then annually 1

Pulmonary Rehabilitation and Exercise

All patients with impaired exercise capacity should participate in pulmonary rehabilitation and take regular exercise. 1, 3

• Duration: 6-8 weeks of supervised exercise training 1
• Benefits: Improved exercise capacity, quality of life, reduced exacerbations, with effects maintained for 3-6 months 1
• Adjunct benefit: May reduce exacerbation frequency (median 1 vs 2 exacerbations over 12 months) 1

Pharmacological Management

Mucoactive Therapy

• Consider humidification with sterile water or normal saline to facilitate airway clearance 1
• Trial mucoactive treatment (such as carbocysteine for 6 months) in patients with difficulty expectorating sputum, only after standard airway clearance techniques have failed 1
• Pre-treatment bronchodilator should be used before inhaled mucoactive drugs to increase tolerability and optimize deposition 1
• Avoid recombinant human DNase - this is strongly contraindicated in non-CF bronchiectasis 1

Bronchodilators

• Do not routinely offer long-acting bronchodilators for bronchiectasis alone 1
• Consider on individual basis for patients with significant breathlessness 1
• Use as indicated for comorbid asthma or COPD according to standard guidelines 1
• Use before physiotherapy and inhaled medications to optimize delivery 1

Inhaled Corticosteroids

• Do not routinely offer inhaled corticosteroids for bronchiectasis without other indications (ABPA, asthma, COPD, inflammatory bowel disease) 1

Management of Exacerbations

When patients present with increased cough, sputum volume or purulence, worsened fatigue, or new symptoms:

• Treat with antibiotics for 14 days based on previous sputum culture results 4, 2
• Oral antibiotics are first-line for most exacerbations 1
• Intravenous antibiotics for clinical deterioration or severe exacerbations 1, 2
• Intensify airway clearance during exacerbations 1

Long-Term Antibiotic Therapy (For High-Risk Patients)

Consider long-term therapy only after optimization of airway clearance and treatment of modifiable underlying causes. 1, 5

Patient Selection Criteria

Long-term antibiotics should be considered for patients with:

• ≥3 exacerbations per year requiring antibiotics, OR 5, 3
• ≥1 hospitalization for exacerbation in the previous year 5

Treatment Options Based on Microbiology

For chronic Pseudomonas aeruginosa infection:

• First-line: Inhaled antibiotics (colistin, gentamicin) 2, 3
• Alternative: Macrolides if inhaled antibiotics contraindicated, not tolerated, or not feasible 5

For non-Pseudomonas patients:

• First-line: Oral macrolides 5
• Azithromycin dosing: 250mg three times weekly OR 500mg three times weekly 5
• Erythromycin dosing: 400mg twice daily 5

Critical Safety Measures Before Starting Macrolides

• ECG required: Contraindicated if QTc >450ms (men) or >470ms (women) 1, 5
• Baseline liver function tests 1
• Screen for NTM infection: Macrolides must never be used as monotherapy in NTM as this causes resistance 5, 4
• Duration: Minimum 6-12 months to assess efficacy 1, 5

Follow-Up and Monitoring

Patients requiring secondary care follow-up include those with: 1

• Chronic Pseudomonas aeruginosa, NTM, or MRSA colonization
• Deteriorating bronchiectasis with declining lung function
• Recurrent exacerbations (≥3 per year)
• Receiving long-term antibiotic therapy
• Associated conditions (rheumatoid arthritis, immunodeficiency, inflammatory bowel disease, PCD, ABPA)
• Advanced disease or considering transplantation

Common Pitfalls to Avoid

• Never use recombinant human DNase - it worsens outcomes in non-CF bronchiectasis 1
• Do not start long-term antibiotics before optimizing airway clearance and treating underlying causes 1, 5
• Do not use macrolides without first screening for NTM infection 5, 4
• Do not prescribe inhaled corticosteroids routinely without comorbid asthma or COPD 1
• Ensure physiotherapy assessment occurs before considering pharmacological interventions 1