What is the recommended treatment approach for bronchiectasis?

Treatment of Bronchiectasis

All adults with bronchiectasis must receive daily airway clearance techniques taught by a trained respiratory physiotherapist as the cornerstone of therapy, with treatment escalation based on exacerbation frequency and microbiology results. 1

Foundation: Airway Clearance Techniques

• Teach all patients with chronic productive cough or difficulty expectorating sputum an airway clearance technique (ACT) by a trained respiratory physiotherapist, performing 10-30 minute sessions once or twice daily. 2, 1

• First-line techniques include the active cycle of breathing or oscillating positive-expiratory-pressure (PEP) devices, incorporating the forced-expiration (huff) maneuver with every session. 1

• Use gravity-assisted positioning (modified postural drainage without head-down tilt) unless contraindicated by gastro-oesophageal reflux. 1

• Review technique within 3 months of initiation and conduct annual reassessments to optimize the regimen. 1

• During hospitalizations for exacerbations, provide daily physiotherapy visits until airway clearance is optimized. 1

Mucoactive Therapy (Adjunct to Airway Clearance)

• Consider adding a mucoactive agent (humidification with sterile water/normal saline, or a 6-month trial of carbocysteine) for patients who continue to have difficulty expectorating sputum despite optimal airway-clearance techniques. 1

• Perform airway-reactivity testing before initiating inhaled mucoactive agents to prevent bronchospasm. 1

• Recombinant human DNase (dornase alfa) must never be used in non-cystic fibrosis bronchiectasis because it worsens outcomes. 2, 1

Management of Acute Exacerbations

• Treat every exacerbation with a 14-day course of antibiotics—this duration is superior to shorter courses in reducing treatment failure. 2, 1, 3

• Select antibiotics based on the most recent sputum culture and sensitivity results; obtain sputum for culture before starting antibiotics whenever possible. 2, 1

• Empiric first-line antibiotics:

  • Amoxicillin 500mg three times daily for Streptococcus pneumoniae or Haemophilus influenzae (beta-lactamase negative). 1
  • Ciprofloxacin 500-750mg twice daily for Pseudomonas aeruginosa. 1, 3

• Patients should keep a supply of appropriate antibiotics at home with a self-management plan for prompt self-initiation of therapy. 1

Eradication of New Pseudomonas aeruginosa Isolation

• Offer eradication therapy when P. aeruginosa is first isolated or re-emerges with clinical deterioration, as P. aeruginosa infection is associated with a three-fold increase in mortality risk, seven-fold increase in hospitalization risk, and one additional exacerbation per year. 1, 4

• First-line eradication: oral ciprofloxacin 500-750mg twice daily for 2 weeks. 1

• Second-line eradication: 2 weeks of intravenous antipseudomonal β-lactam ± aminoglycoside, followed by 3 months of nebulized colistin, gentamicin, or tobramycin. 1

• Do not attempt eradication for pathogens other than P. aeruginosa. 1

Long-Term Antibiotic Prophylaxis (≥3 Exacerbations per Year)

Before initiating long-term antibiotics, optimize airway clearance and treat modifiable underlying causes. 2, 3

For Chronic Pseudomonas aeruginosa Infection:

• First-line: long-term inhaled colistin 1MU twice daily via I-neb. 1, 3

• Second-line: inhaled gentamicin if colistin is unsuitable. 1

• Prior inhalation of a short-acting bronchodilator is advisable to prevent bronchospasm (10-32% risk). 2

• Conduct a supervised test dose with pre- and post-spirometry before initiating inhaled antibiotics. 2

For Patients Without Pseudomonas aeruginosa Infection:

• Long-term macrolide therapy (azithromycin 250mg three times weekly or erythromycin) after confirming the absence of nontuberculous mycobacterial infection, as macrolide monotherapy can increase the risk of macrolide resistance in NTM. 2, 1, 3

• If inhaled antibiotics are contraindicated or not tolerated in P. aeruginosa patients, consider long-term macrolide therapy. 1

Monitoring Requirements:

• Careful characterization of sputum pathogens (bacteria, mycobacteria, fungi) before and after implementation of long-term antibiotics is essential to direct antibiotic choices, monitor resistance patterns, and identify treatment-emergent organisms. 2

• Drug toxicity monitoring is required, most notably with macrolides and inhaled aminoglycosides. 2

Bronchodilator Therapy

• Provide a trial of long-acting bronchodilators (LABA, LAMA, or combination) for patients with significant breathlessness, especially those with chronic airflow limitation. 1

• Administer bronchodilators before physiotherapy sessions and before inhaled antibiotics to improve pulmonary drug deposition. 1

• Discontinue bronchodilator therapy if no symptomatic improvement is observed. 1

Anti-Inflammatory Treatments

• Inhaled corticosteroids should not be routinely prescribed unless the patient has comorbid asthma or COPD. 1, 3

• Long-term oral corticosteroids should not be offered without other indications such as ABPA, chronic asthma, COPD, or inflammatory bowel disease. 1

• Statins are not recommended for bronchiectasis treatment. 1

Pulmonary Rehabilitation & Exercise

• All patients with impaired exercise capacity should enroll in a supervised 6-8 week pulmonary rehabilitation program—this strong recommendation is supported by high-quality evidence showing improvements in exercise capacity, cough symptoms, quality of life, and a reduction in exacerbation frequency. 2, 1

• Encourage regular physical exercise combined with the forced-expiration technique to further promote airway clearance. 1

Immunizations

• Offer annual influenza vaccination to all patients with bronchiectasis. 1, 3

• Offer pneumococcal vaccination (23-valent pneumococcal polysaccharide vaccine) to all patients. 1

• In patients who do not achieve an adequate serologic response to the polysaccharide vaccine, consider a 13-valent pneumococcal conjugate vaccine. 1

• Consider influenza vaccination in household contacts of patients with immune deficiency and bronchiectasis. 1

Diagnostic Work-up

• After confirming bronchiectasis on high-resolution CT, obtain a minimum test bundle that includes differential blood count, serum immunoglobulin levels (IgG, IgA, IgM), testing for allergic bronchopulmonary aspergillosis, and sputum culture for bacteria and mycobacteria at every clinical visit. 1, 3

• Perform bronchoscopy with bronchial aspiration or wash in patients with localized disease or who cannot expectorate sputum, to exclude endobronchial lesions, foreign bodies, and to diagnose nontuberculous mycobacterial infection. 1

Surgical Considerations

• Surgery is not recommended except in cases of localized disease with high exacerbation frequency despite optimization of all other management aspects. 2, 1, 3

• Video-assisted thoracoscopic surgery (VATS) is often preferred to better preserve lung function and reduce scarring compared to open surgery. 1

• Emergency surgery in unstable patients with massive hemoptysis is associated with higher morbidity and mortality reaching 37%. 1

Lung Transplantation Referral

• Refer patients ≤65 years for lung transplantation when FEV₁ <30% with significant clinical instability or rapid progressive respiratory decline despite optimal medical therapy, and also for factors such as massive hemoptysis, severe secondary pulmonary hypertension, ICU admissions, or respiratory failure. 1, 3

Critical Pitfalls to Avoid

• Never extrapolate treatments from cystic fibrosis bronchiectasis, as treatment responses differ. 1, 3

• Do not use antibiotic courses shorter than 14 days for exacerbations, as this increases treatment failure risk. 3

• Failure to identify and treat P. aeruginosa infection aggressively is a major pitfall given its dramatic impact on outcomes. 4

• Inadequate etiological workup can miss treatable causes like immunodeficiency or ABPA. 4

• Underutilization of airway clearance techniques and pulmonary rehabilitation despite strong evidence for their benefit. 4