What is the recommended management approach for patients with bronchiectasis?

Management of Bronchiectasis in Adults

All patients with bronchiectasis should receive annual influenza and pneumococcal vaccinations, be taught airway clearance techniques by a trained respiratory physiotherapist, and be considered for long-term antibiotic therapy if experiencing ≥3 exacerbations per year. 1, 2

Initial Diagnostic Workup

• Confirm diagnosis with high-resolution CT (HRCT) scanning, which is the gold standard for demonstrating permanent bronchial dilatation 2
• Obtain differential blood count, serum immunoglobulins, testing for allergic bronchopulmonary aspergillosis (ABPA), and sputum culture for bacteria, mycobacteria, and fungi 2
• Assess for chronic rhinosinusitis symptoms, as this commonly coexists with bronchiectasis 1
• Perform pulse oximetry at each visit to screen for respiratory failure 1

Non-Pharmacological Management

Airway Clearance Techniques

All patients with chronic productive cough or difficulty expectorating sputum must be taught airway clearance techniques by a trained respiratory physiotherapist. 2, 3

• Sessions should last 10-30 minutes, once or twice daily 2, 3
• Techniques include active cycle of breathing, postural drainage, and manual or mechanical devices 2
• Consider intermittent positive pressure breathing or non-invasive ventilation during acute exacerbations to reduce work of breathing 3

Pulmonary Rehabilitation

Strongly recommend pulmonary rehabilitation for all patients with impaired exercise capacity. 2, 4

• Provides improved exercise capacity, reduced cough symptoms, enhanced quality of life, and decreased frequency of exacerbations 2
• Annual assessment by respiratory physiotherapist to optimize airway clearance regimen 2

Mucoactive Treatments

• Consider long-term mucoactive treatment for patients with difficulty expectorating sputum, poor quality of life, or failure of standard airway clearance techniques 2, 3
• Consider humidification with sterile water or normal saline to facilitate airway clearance 3
• Do NOT use recombinant human DNase (dornase alfa) in non-CF bronchiectasis 2, 3

Pharmacological Management

Acute Exacerbations

Treat all exacerbations with 14 days of antibiotics, selected based on previous sputum culture results. 1, 2, 3

• Obtain sputum cultures before starting antibiotics whenever possible 3
• Common pathogens and first-line antibiotics: 3
  • Streptococcus pneumoniae: Amoxicillin 500mg TID for 14 days
  • Haemophilus influenzae (beta-lactamase negative): Amoxicillin 500mg TID for 14 days
  • Pseudomonas aeruginosa: Ciprofloxacin 500-750mg BID for 14 days
• Consider intravenous antibiotics for patients who are particularly unwell, have resistant organisms, or have failed oral therapy (most likely with P. aeruginosa) 1, 3

Long-Term Antibiotic Therapy

Consider long-term antibiotics for patients with ≥3 exacerbations per year. 2, 3, 4

• First-line for chronic Pseudomonas aeruginosa infection: Long-term inhaled antibiotics (nebulised colistin, gentamicin, or tobramycin) 1, 2, 4
• First-line for patients WITHOUT Pseudomonas aeruginosa: Macrolides (azithromycin or clarithromycin) 2, 3, 4
• P. aeruginosa infection is associated with three-fold increase in mortality risk, almost seven-fold increase in risk of hospital admission, and an average of one additional exacerbation per patient per year 3

Pseudomonas Aeruginosa Eradication

Offer eradication antibiotic treatment for new growth of P. aeruginosa (first isolation or regrowth) associated with clinical deterioration. 1

• First-line: Ciprofloxacin 500-750mg BID for 2 weeks 1
• Second-line: IV antipseudomonal beta-lactam ± IV aminoglycoside for 2 weeks, followed by 3-month course of nebulised colistin, gentamicin, or tobramycin 1
• Discuss risks and benefits of eradication treatment versus clinical observation with patients 1

MRSA Eradication

• Offer eradication treatment for new growth of methicillin-resistant S. aureus (MRSA), especially in context of clinical deterioration 1

Bronchodilator Therapy

• Do NOT routinely prescribe bronchodilators for all patients 2
• Consider long-acting bronchodilators only for patients with significant breathlessness on an individual basis 2
• If treatment does not reduce symptoms, discontinue it 3

Anti-Inflammatory Treatments

Do NOT routinely offer inhaled corticosteroids unless comorbid asthma or COPD is present. 2, 3, 4

• Do NOT offer long-term oral corticosteroids without other indications such as ABPA, chronic asthma, COPD, or inflammatory bowel disease 3
• For active ABPA: Oral corticosteroid at initial dose of 0.5 mg/kg/day for 2 weeks, weaning according to clinical response and serum IgE levels 1
• Consider itraconazole as steroid-sparing agent for patients dependent on oral corticosteroids with difficulty weaning 1
• Monitor patients with active ABPA using total IgE level to assess treatment response 1

Immunizations

Offer annual influenza immunization to ALL patients with bronchiectasis. 1, 3

Offer polysaccharide pneumococcal vaccination (23-valent) to ALL patients with bronchiectasis. 1, 3

• Consider influenza vaccination in household contacts of patients with immune deficiency and bronchiectasis to reduce secondary transmission 1
• Consider 13-valent protein conjugate pneumococcal vaccine for patients without appropriate serological response to standard polysaccharide vaccine 1

Monitoring and Follow-Up

All patients require routine monitoring to identify disease progression, pathogen emergence, and modify treatment. 1

• Tailor frequency of monitoring to disease severity, with annual assessment minimum and more frequent in severe disease 1
• Record patient's weight and BMI at each clinic appointment 1
• Perform pulse oximetry to screen for patients who may need blood gas analysis to detect respiratory failure 1
• Regular monitoring of sputum pathogens, especially when using long-term antibiotics 2
• Monitor for drug toxicity, particularly with macrolides and inhaled aminoglycosides 2
• Baseline chest X-ray may provide useful comparator in event of clinical deterioration 1

Management of Comorbidities

• Assess for symptoms of chronic rhinosinusitis and treat according to evidence-based pathways 1
• Consider trial of inhaled and/or oral corticosteroids in patients with inflammatory bowel disease (IBD) 1
• Ensure optimal control of asthma and allergies in patients with both bronchiectasis and asthma 1
• Monitor patients with co-morbid COPD and bronchiectasis closely as they are at higher risk of death 1
• Patients with autoimmune conditions require careful assessment for autoimmune-related lung disease and long-term secondary care follow-up 1

Advanced Interventions

Respiratory Support

• Consider long-term oxygen therapy for patients with respiratory failure, using same eligibility criteria as for COPD 1
• Consider domiciliary non-invasive ventilation with humidification for patients with respiratory failure associated with hypercapnia, especially with symptoms or recurrent hospitalisation 1

Surgical Intervention

Consider lung resection ONLY in patients with localized disease whose symptoms are not controlled by medical treatment optimized by a bronchiectasis specialist. 1, 2

• Offer multidisciplinary assessment including bronchiectasis physician, thoracic surgeon, and experienced anaesthetist for suitability evaluation and pre-operative cardiopulmonary reserve assessment 1

Lung Transplantation

Consider transplant referral in patients aged ≤65 years if FEV₁ <30% with significant clinical instability or rapid progressive respiratory deterioration despite optimal medical management. 1, 3

• Consider earlier referral with additional factors: massive haemoptysis, severe secondary pulmonary hypertension, ICU admissions, or respiratory failure (particularly requiring NIV) 1, 3
• Discuss appropriate patients with transplant centre prior to formal referral 1
• Optimize comorbidities such as osteoporosis and maintain physical condition through pulmonary rehabilitation prior to transplant 1

Infection Control

• Individual or cohort segregation based on respiratory tract microbiology results is NOT routinely required 1
• Apply good cross-infection prevention principles according to local policies 1
• In shared facilities with cystic fibrosis patients, cystic fibrosis cross-infection guidelines should prevail 1

Common Pitfalls and Caveats

• Do NOT extrapolate treatments from cystic fibrosis bronchiectasis, as treatment responses are different 3
• Antibiotic sensitivity testing should guide treatment, but some patients may respond despite in vitro resistance—only change antibiotics if no clinical response 1
• Shorter antibiotic courses may suffice in patients with mild bronchiectasis, but always use 14 days for P. aeruginosa infections 1
• For haemoptysis ≤10 mls over 24 hours, treat with appropriate oral antibiotic; if clinical deterioration occurs, arrange emergency hospital admission 1