What is the recommended treatment approach for a patient diagnosed with bronchiectasis?

Treatment of Bronchiectasis

All patients with bronchiectasis should receive airway clearance techniques taught by a trained respiratory physiotherapist, performed once or twice daily for 10-30 minutes, and those with impaired exercise capacity should participate in pulmonary rehabilitation programs. 1, 2

Core Treatment Algorithm

Step 1: Non-Pharmacological Foundation (All Patients)

Airway Clearance Techniques:

• Every patient with chronic productive cough or difficulty expectorating must be taught airway clearance techniques by a trained respiratory physiotherapist 1, 2
• Sessions should last 10-30 minutes, performed once or twice daily 1, 2
• Techniques include chest percussion, vibration, postural drainage, and oscillatory devices 1, 3

Pulmonary Rehabilitation:

• Strongly recommended for all patients with impaired exercise capacity 1, 2
• Consists of 6-8 weeks of supervised exercise training 2
• Improves exercise capacity, reduces cough symptoms, enhances quality of life, and decreases exacerbation frequency 1, 2

Immunizations:

• Annual influenza vaccination for all patients 1, 2
• Pneumococcal vaccination for all patients 1, 2

Step 2: Treat Acute Exacerbations

Antibiotic Duration:

• Always treat exacerbations with 14 days of antibiotics (not the typical 7-10 days used for other respiratory infections) 1, 2
• Obtain sputum cultures before starting antibiotics whenever possible 1, 2

Antibiotic Selection Based on Pathogen:

• Streptococcus pneumoniae: Amoxicillin 500mg three times daily for 14 days 2
• Haemophilus influenzae (beta-lactamase negative): Amoxicillin 500mg three times daily for 14 days 2
• Pseudomonas aeruginosa: Ciprofloxacin 500-750mg twice daily for 14 days 1, 2
• Consider intravenous antibiotics for patients who are particularly unwell, have resistant organisms, or have failed oral therapy (most likely with P. aeruginosa) 1, 2

Step 3: Long-Term Antibiotic Prophylaxis (Only for Frequent Exacerbators)

Indication:

• Only consider for patients with ≥3 exacerbations per year 1, 2
• Must first optimize airway clearance and treat modifiable underlying causes 1

Treatment Selection Based on Pseudomonas aeruginosa Status:

If Chronic P. aeruginosa Infection Present:

• First-line: Long-term inhaled antibiotics (colistin or gentamicin) 1, 2, 4
• P. aeruginosa infection is associated with 3-fold increase in mortality, 7-fold increase in hospital admission risk, and one additional exacerbation per patient per year 2, 5
• Second-line: Macrolides (azithromycin or erythromycin) if inhaled antibiotics are contraindicated, not tolerated, or not feasible 1
• Consider adding macrolides to inhaled antibiotics if high exacerbation frequency persists 1

If No P. aeruginosa Infection:

• First-line: Long-term macrolides (azithromycin or erythromycin) 1, 2, 4
• Critical caveat: Must exclude active nontuberculous mycobacterial (NTM) infection before starting macrolides 3, 4
• Second-line: Long-term oral antibiotics (choice based on antibiotic susceptibility) if macrolides are contraindicated, not tolerated, or ineffective 1
• Third-line: Long-term inhaled antibiotics if oral antibiotics are contraindicated, not tolerated, or ineffective 1

Step 4: Mucoactive Treatments (Selective Use)

Indications:

• Consider for patients with difficulty expectorating sputum, poor quality of life, or failure of standard airway clearance techniques 1, 2
• Options include nebulized hypertonic saline or mannitol 1, 2
• Hypertonic saline 7% showed improvement in FEV1 and FVC at 3 months 1

Critical Contraindication:

• Never use recombinant human DNase (dornase alfa) in non-CF bronchiectasis - it may cause harm 1, 3
• This is a dangerous example of extrapolating from cystic fibrosis, where treatment responses are different 2, 5

Step 5: Bronchodilators (Selective Use)

Not Routinely Recommended:

• Do not routinely offer long-acting bronchodilators for all patients with bronchiectasis 1

Specific Indications:

• Offer trial of long-acting bronchodilators (LABA, LAMA, or combination) only for patients with significant breathlessness, particularly those with chronic obstructive airflow limitation 1, 2, 3
• Use bronchodilators before physiotherapy, inhaled mucoactive drugs, and inhaled antibiotics to increase tolerability and optimize pulmonary deposition 1
• Discontinue if treatment does not reduce symptoms 2, 3

Comorbid Conditions:

• The diagnosis of bronchiectasis should not affect the use of bronchodilators in patients with comorbid asthma or COPD 1
• Follow COPD or asthma guideline recommendations for these patients 2

Step 6: Inhaled Corticosteroids (Generally Not Recommended)

Do not routinely offer inhaled corticosteroids unless comorbid asthma or COPD is present 1, 2, 3

Exceptions:

• Use according to asthma or COPD guidelines if these conditions coexist 1
• Consider for patients with allergic bronchopulmonary aspergillosis (ABPA): oral corticosteroid 0.5 mg/kg/day for 2 weeks initially, weaned according to clinical response and serum IgE levels 1
• Consider trial for patients with inflammatory bowel disease 1

Step 7: Eradication Treatment for New P. aeruginosa Isolation

Offer eradication antibiotic treatment to adults with new isolation of P. aeruginosa 1

Do not offer eradication treatment for pathogens other than P. aeruginosa 1

Step 8: Surgical Considerations (Rare)

Surgery is NOT recommended except in highly selected cases 1

Strict Criteria for Surgical Consideration:

• Only for patients with truly localized disease (not multilobar) 1, 2, 5
• High exacerbation frequency despite optimization of ALL other aspects of management 1, 2
• Surgical mortality rate is 1.4% with post-operative morbidity of 16.2% 5
• Video-assisted thoracoscopic surgery (VATS) is preferred over open surgery to preserve lung function 2

Lung Transplantation:

• Consider for patients aged ≤65 years if FEV1 <30% with significant clinical instability or rapid progressive respiratory deterioration despite optimal medical management 1, 2, 5
• Consider earlier referral with massive hemoptysis, severe secondary pulmonary hypertension, ICU admissions, or respiratory failure 1, 2

Essential Diagnostic Workup

Minimum Bundle of Etiological Tests:

• Differential blood count 1
• Serum immunoglobulins (total IgG, IgA, IgM) 1
• Testing for allergic bronchopulmonary aspergillosis 1
• Sputum culture for bacterial infection monitoring 1
• Mycobacterial culture in selected cases where NTM is suspected 1

High-resolution CT (HRCT) is the diagnostic procedure of choice to confirm bronchiectasis 1, 3

Critical Pitfalls to Avoid

Pitfall #1: Inadequate Antibiotic Duration

• Treating exacerbations with less than 14 days increases treatment failure risk 2, 5

Pitfall #2: Starting Long-Term Antibiotics Too Early

• Must first optimize airway clearance and treat underlying causes before considering prophylactic antibiotics 1

Pitfall #3: Using Dornase Alfa

• This helps CF patients but may harm non-CF bronchiectasis patients 1, 3

Pitfall #4: Starting Macrolides Without Excluding NTM

• Always exclude active NTM infection before starting macrolides to avoid monotherapy resistance 3, 4

Pitfall #5: Underutilizing Airway Clearance and Pulmonary Rehabilitation

• These have the strongest evidence base but are commonly underused 2, 5

Pitfall #6: Failing to Aggressively Manage P. aeruginosa

• This pathogen dramatically worsens outcomes and requires aggressive treatment 2, 5

Pitfall #7: Considering Surgery for Multilobar Disease

• Removing multiple lobes causes unacceptable loss of lung function 5

Monitoring Strategy

Regular Monitoring Required:

• Tailor frequency to disease severity (annual for mild disease, more frequent for severe) 1
• Monitor sputum pathogens, especially when using long-term antibiotics 1, 2, 3
• Monitor for drug toxicity, particularly with macrolides and inhaled aminoglycosides 2, 3
• Pulse oximetry to screen for respiratory failure 1
• Annual assessment by respiratory physiotherapist to optimize airway clearance regimen 5
• Breathlessness is one of the strongest predictors of mortality and should trigger therapy intensification 5