What is the recommended treatment plan for a patient with bronchiectasis?

Treatment of Bronchiectasis

All patients with bronchiectasis should receive airway clearance techniques taught by a trained respiratory physiotherapist, with sessions lasting 10-30 minutes once or twice daily, and those with ≥3 exacerbations per year should be offered long-term antibiotics (inhaled antibiotics for chronic Pseudomonas aeruginosa infection, or macrolides for non-Pseudomonas patients). 1, 2

Core Treatment Components

The management of bronchiectasis targets four key pathophysiological features: chronic bronchial infection, inflammation, impaired mucociliary clearance, and structural lung damage. 2

Airway Clearance (Foundation for All Patients)

• All patients with chronic productive cough or difficulty expectorating should receive instruction from a trained respiratory physiotherapist in airway clearance techniques, performed 10-30 minutes, once or twice daily. 1, 2
• Consider intermittent positive pressure breathing or non-invasive ventilation during acute exacerbations to reduce work of breathing. 2
• Consider long-term mucoactive treatment (such as nebulized hypertonic saline or normal saline) for patients with difficulty expectorating sputum, poor quality of life, or failure of standard airway clearance techniques. 1, 2
• Do not routinely use recombinant human DNase (dornase alfa) in adults with non-CF bronchiectasis, as it may worsen outcomes. 1, 2

Pulmonary Rehabilitation and Exercise

• Patients with impaired exercise capacity should participate in pulmonary rehabilitation programs consisting of 6-8 weeks of supervised exercise training, which improves exercise capacity, reduces cough symptoms, enhances quality of life, and decreases exacerbation frequency. 2, 3
• All patients should be encouraged to take regular exercise. 2

Bronchodilator Therapy

• Offer a trial of long-acting bronchodilator therapy (LABA, LAMA, or combination) in patients with significant breathlessness, particularly those with chronic obstructive airflow limitation. 2
• Perform reversibility testing to identify co-existing asthma, though it is not required to predict benefit from bronchodilators. 2
• If bronchodilators do not reduce symptoms, discontinue them. 2

Antibiotic Management: Stepwise Approach

Step 1: Acute Exacerbations

Treat all exacerbations with 14 days of antibiotics, selected based on previous sputum culture results. 1, 2, 4

Obtain sputum for culture and sensitivity before starting antibiotics whenever possible. 1, 4

Common pathogens and first-line oral antibiotics: 1, 2

• Haemophilus influenzae (beta-lactamase negative): Amoxicillin 500mg three times daily for 14 days
• Streptococcus pneumoniae: Amoxicillin 500mg three times daily for 14 days
• Pseudomonas aeruginosa: Ciprofloxacin 500-750mg twice daily for 14 days
• Patients without risk factors for P. aeruginosa: Amoxicillin-clavulanate 625mg three times daily for 14 days

Consider intravenous antibiotics when patients are particularly unwell, have resistant organisms, or have failed to respond to oral therapy (most likely with P. aeruginosa). 1, 4

Intravenous options for P. aeruginosa: 4

• Ceftazidime 2g three times daily
• Piperacillin-tazobactam 4.5g three times daily
• Meropenem 2g three times daily

Step 2: Pseudomonas Aeruginosa Eradication

For new growth of P. aeruginosa (first isolation or regrowth), offer eradication treatment: 1

• First-line: Ciprofloxacin 500-750mg twice daily for 2 weeks
• Second-line: IV antipseudomonal beta-lactam ± IV aminoglycoside for 2 weeks, followed by 3 months of nebulized colistin, gentamicin, or tobramycin

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. 2

Step 3: Long-term Antibiotics (≥3 Exacerbations/Year)

Consider long-term antibiotics for patients with ≥3 exacerbations per year despite optimal airway clearance. 1, 2, 3

For patients with chronic P. aeruginosa infection: 1, 2

• First-line: Inhaled colistin (nebulized)
• Second-line: Inhaled gentamicin as alternative to colistin
• Alternative or additive: Azithromycin or erythromycin (if patient does not tolerate inhaled antibiotics, or as additive treatment for high exacerbation frequency)

For patients without P. aeruginosa infection: 1, 2, 3

• Macrolides: Azithromycin or erythromycin

Safety precautions before starting long-term antibiotics: 1

Before macrolides:

• Ensure no active NTM infection with at least one negative respiratory NTM culture
• Use with caution if significant hearing loss needing hearing aids or significant balance issues

Before inhaled aminoglycosides:

• Avoid if creatinine clearance <30ml/min
• Use with caution if significant hearing loss needing hearing aids or significant balance issues
• Avoid concomitant nephrotoxic medications

Anti-inflammatory Therapy

• Do not routinely offer inhaled corticosteroids unless comorbid asthma or COPD is present. 1, 2, 3
• Do not routinely offer long-term oral corticosteroids without other indications. 1, 2
• Do not routinely offer PDE4 inhibitors, methylxanthines, leukotriene receptor antagonists, CXCR2 antagonists, neutrophil elastase inhibitors, or statins for bronchiectasis treatment. 1

Special Circumstances

For allergic bronchopulmonary aspergillosis (ABPA): 1, 2

• Offer oral corticosteroid at initial dose of 0.5mg/kg/day for 2 weeks
• Wean steroids according to clinical response and serum IgE levels
• Monitor with total IgE level to assess treatment response
• Consider itraconazole as steroid-sparing agent for patients dependent on oral corticosteroids

For inflammatory bowel disease: Consider trial of inhaled and/or oral corticosteroids. 1

For asthma comorbidity: Ensure optimal control of asthma and allergies. 1

Monitoring and Follow-up

• All patients should undergo routine monitoring to identify disease progression, pathogen emergence, and modify treatment. 1
• Tailor frequency of monitoring to disease severity: assess patients annually, and more frequently in severe disease. 1
• Perform pulse oximetry to screen for patients who may need blood gas analysis to detect respiratory failure. 1
• Send sputum for culture and sensitivity immediately before and at each clinical visit after antibiotics to determine treatment outcome. 1, 4

Immunizations

• Offer annual influenza immunization to all patients. 2
• Offer pneumococcal vaccination to all patients. 2
• Consider influenza vaccination in household contacts of patients with immune deficiency. 2

Surgical Considerations

• Surgery should be limited to patients with localized disease who have not responded to maximal medical therapy and have intolerable symptoms. 1, 2
• Video-assisted thoracoscopic surgery (VATS) is often preferred to better preserve lung function and reduce scarring. 2
• Consider transplant referral in patients aged ≤65 years if FEV1 <30% with significant clinical instability, rapid progressive respiratory deterioration despite optimal medical management, massive hemoptysis, severe secondary pulmonary hypertension, ICU admissions, or respiratory failure. 2

Critical Pitfalls to Avoid

• Never use antibiotic courses shorter than 14 days when P. aeruginosa is present. 1, 4
• Never initiate long-term macrolides without ruling out active NTM infection with at least one negative respiratory culture. 1, 4
• Do not change antibiotics for in vitro resistance if patient is responding clinically—some patients respond despite laboratory resistance. 1, 4
• Do not extrapolate treatments from cystic fibrosis bronchiectasis, as treatment responses are different. 2
• Patients with co-morbid COPD and bronchiectasis are at higher risk of death and require closer monitoring. 1