What are the guidelines for managing a patient with bronchiectasis?

Guidelines for Bronchiectasis Management in Adults

All patients with bronchiectasis should receive airway clearance techniques taught by a trained respiratory physiotherapist, with sessions performed once or twice daily for 10-30 minutes, and those with ≥3 exacerbations per year should be considered for long-term antibiotic therapy (inhaled antibiotics for chronic Pseudomonas aeruginosa infection or macrolides for non-Pseudomonas infection). 1

Initial Assessment and Monitoring

• Obtain sputum for culture and sensitivity testing at diagnosis and before starting antibiotics during exacerbations to guide treatment selection 1
• Assess disease severity annually at minimum, with more frequent monitoring (every 3-6 months) in patients with severe disease or frequent exacerbations 1
• Perform pulse oximetry at each visit to screen for respiratory failure requiring blood gas analysis 1
• Evaluate for underlying causes including immunodeficiency, ABPA, autoimmune conditions, and primary ciliary dyskinesia 1, 2
• Screen for chronic rhinosinusitis as this commonly coexists with bronchiectasis and requires treatment 1

Airway Clearance (Non-Pharmacological Foundation)

• Teach all patients with chronic productive cough an airway clearance technique by a trained respiratory physiotherapist 1, 3
• Sessions should last 10-30 minutes, performed once or twice daily using techniques such as active cycle of breathing, autogenic drainage, or oscillating positive expiratory pressure devices 1, 3
• Consider mucoactive agents (hypertonic saline or mannitol) for patients with difficulty expectorating despite standard airway clearance techniques and poor quality of life 1, 3
• Do NOT use recombinant human DNase (dornase alfa) in non-CF bronchiectasis as it may worsen outcomes 1, 3

Pulmonary Rehabilitation

• All patients with impaired exercise capacity should participate in pulmonary rehabilitation consisting of 6-8 weeks of supervised exercise training 1, 3, 2
• This intervention has strong evidence for improving exercise capacity, reducing cough symptoms, enhancing quality of life, and decreasing exacerbation frequency 1, 2

Management of Acute Exacerbations

• Treat all exacerbations with 14 days of antibiotics, selecting based on previous sputum culture results 1, 4, 3
• Obtain sputum for culture before starting antibiotics whenever possible, but do not delay treatment if sputum cannot be obtained 1, 4
• Start empirical antibiotics while awaiting culture results, then modify based on sensitivity testing if no clinical improvement 1

Antibiotic Selection by Pathogen:

• Streptococcus pneumoniae or Haemophilus influenzae (beta-lactamase negative): Amoxicillin 500mg three times daily for 14 days 3
• Pseudomonas aeruginosa: Ciprofloxacin 500-750mg twice daily for 14 days (first-line oral therapy) 1, 4, 3
• Consider intravenous antibiotics when patients are particularly unwell, have resistant organisms, or have failed oral therapy (most commonly with P. aeruginosa infection) 1

Long-Term Antibiotic Therapy for Prevention

Indications:

• Consider long-term antibiotics for patients with ≥3 exacerbations per year despite optimized airway clearance 1, 4, 3

For Chronic Pseudomonas aeruginosa Infection:

• First-line: Long-term inhaled antibiotics (colistin, gentamicin, or tobramycin) 1, 4, 5, 2
• Second-line: Add or substitute macrolides (azithromycin or erythromycin) if inhaled antibiotics alone are insufficient 1
• P. aeruginosa infection is associated with three-fold increase in mortality, seven-fold increase in hospital admission risk, and one additional exacerbation per year 3

For Non-Pseudomonas Infection:

• First-line: Long-term macrolides (azithromycin or erythromycin) 1, 3, 2
• Monitor for adverse effects including hearing loss, QT prolongation, and development of macrolide resistance 6
• Consider alternative oral antibiotics (based on sensitivity testing) if macrolides are contraindicated, not tolerated, or ineffective 1

Pseudomonas aeruginosa Eradication Strategy:

• Offer eradication treatment for first isolation or regrowth of P. aeruginosa 1, 4, 5
• First-line eradication: Ciprofloxacin 500-750mg twice daily for 2 weeks 1, 4, 5
• Second-line eradication: IV antipseudomonal beta-lactam ± aminoglycoside for 2 weeks, followed by 3 months of nebulized colistin, gentamicin, or tobramycin 1

Bronchodilator Therapy

• Do NOT routinely offer long-acting bronchodilators unless the patient has comorbid asthma or COPD 1, 3
• Consider a trial of bronchodilators (LABA, LAMA, or combination) in patients with significant breathlessness on an individual basis 1, 3
• Use bronchodilators before physiotherapy and inhaled antibiotics to optimize pulmonary deposition and tolerability 1
• Discontinue if no symptom improvement after trial period 3

Anti-Inflammatory Therapy

• Do NOT routinely offer inhaled corticosteroids unless comorbid asthma or COPD is present 3, 2
• Do NOT offer long-term oral corticosteroids without specific indications (ABPA, chronic asthma, COPD, or inflammatory bowel disease) 3
• For active ABPA: Offer oral corticosteroids at 0.5 mg/kg/day for 2 weeks, then wean according to clinical response and serum IgE levels 1

Management of Comorbidities

• Monitor patients with comorbid COPD closely as they are at higher risk of death 1
• Ensure optimal control of asthma and allergies in patients with both conditions 1
• Consider trial of inhaled/oral corticosteroids in patients with inflammatory bowel disease 1
• Refer patients requiring DMARDs or biologics for rheumatoid arthritis to a chest physician before starting treatment 1

Immunizations

• Offer annual influenza vaccination to all patients with bronchiectasis 1, 4, 3
• Offer pneumococcal vaccination (23-valent polysaccharide vaccine) to all patients 1, 4, 3
• Consider 13-valent conjugate vaccine in patients without appropriate serological response to standard polysaccharide vaccine 1
• Consider influenza vaccination for household contacts of patients with immune deficiency to reduce secondary transmission 1, 3

Advanced Therapies

Long-Term Oxygen Therapy:

• Consider LTOT for patients with respiratory failure using the same eligibility criteria as COPD 1, 5

Non-Invasive Ventilation:

• Consider domiciliary NIV with humidification for patients with respiratory failure associated with hypercapnia, especially with symptoms or recurrent hospitalization 1

Surgical Resection:

• Consider lung resection ONLY in patients with localized disease whose symptoms are not controlled despite optimized medical treatment by a bronchiectasis specialist 1, 5, 3
• Require multidisciplinary assessment including bronchiectasis physician, thoracic surgeon, and experienced anesthetist 1
• Video-assisted thoracoscopic surgery (VATS) is preferred over open surgery to preserve lung function 3

Lung Transplantation:

• Consider transplant referral in patients ≤65 years if FEV₁ <30% with significant clinical instability or rapid progressive deterioration despite optimal management 1, 4, 3
• Consider earlier referral with additional factors: massive hemoptysis, severe secondary pulmonary hypertension, ICU admissions, or respiratory failure requiring NIV 1, 3

Infection Control

• Individual or cohort segregation is NOT routinely required based on respiratory microbiology results 1
• Apply good cross-infection prevention principles according to local policies 1
• In shared facilities with CF patients, follow CF cross-infection guidelines as P. aeruginosa transmissibility appears more common in CF 1

Common Pitfalls and Caveats

• Shorter antibiotic courses (<14 days) may suffice only in patients with mild bronchiectasis without P. aeruginosa infection, but 14 days should ALWAYS be used for P. aeruginosa 1
• Some patients may respond to antibiotics despite in vitro resistance—only change antibiotics if there is no clinical response 1
• Repeat sensitivity testing in patients on long-term antibiotics if there is clinical concern regarding loss of efficacy 1
• Do NOT extrapolate treatments from CF bronchiectasis as treatment responses differ significantly 3
• Macrolide resistance and adverse effects (hearing loss, QT prolongation) require ongoing monitoring in patients on long-term therapy 6

1, 4, 5, 3, 2