What are the recommended management strategies for an adult patient with bronchiectasis?

Management of Adult Bronchiectasis

All adults with bronchiectasis must perform daily airway clearance techniques taught by a respiratory physiotherapist, with treatment escalation based on exacerbation frequency and microbiology results. 1, 2

Initial Diagnostic Workup

When bronchiectasis is confirmed on high-resolution CT, obtain the following minimum bundle of tests 1, 3:

• Differential blood count 1
• Serum immunoglobulins (total IgG, IgA, IgM) 1
• Testing for allergic bronchopulmonary aspergillosis (ABPA) 1, 3
• Sputum culture for bacteria and mycobacteria at every clinical visit to guide antibiotic selection 1, 3
• Serum protein electrophoresis if immunoglobulins are elevated 1

Consider bronchoscopy with bronchial aspiration or wash for patients with localized disease to exclude endobronchial lesions or foreign bodies, and for those who cannot expectorate sputum, particularly to diagnose nontuberculous mycobacterial disease 1.

Foundation: Airway Clearance Techniques (Universal for All Patients)

Every patient with chronic productive cough or difficulty expectorating must receive instruction from a trained respiratory physiotherapist 1, 2. This is the cornerstone of management and takes priority over pharmacological interventions 2.

Specific Techniques to Teach

• First-line options: Active cycle of breathing techniques OR oscillating positive expiratory pressure devices 1, 2
• Duration: 10-30 minutes, once or twice daily, until two clear huffs or coughs are completed 2, 3
• Adjuncts: Incorporate the forced expiration technique (huff) with all airway clearance methods 1
• Positioning: Use gravity-assisted positioning (modified postural drainage without head-down tilt) unless contraindicated by gastroesophageal reflux 1

Alternative Techniques

If standard methods are ineffective or unacceptable, consider autogenic drainage, positive expiratory pressure, high-frequency chest wall oscillation, or intrapulmonary percussive ventilation 1.

Follow-up Schedule

• Review technique within 3 months of initial assessment 2
• Annual reassessment by respiratory physiotherapist to optimize regimen 1, 3
• Daily physiotherapy visits during hospitalizations for exacerbations until airway clearance is optimized 1

Mucoactive Therapy (Adjunct to Airway Clearance)

Add mucoactive agents only for patients with persistent difficulty expectorating despite optimal airway clearance techniques 2, 3:

• Humidification with sterile water or normal saline to facilitate clearance 2, 3
• Carbocysteine: Trial for 6 months; continue only if clinical benefit observed 2
• Perform airway reactivity testing before initiating any inhaled mucoactive agents 2

Critical Contraindication

Never use recombinant human DNase (dornase alfa) in non-cystic fibrosis bronchiectasis—it worsens outcomes 1, 2, 3.

Pulmonary Rehabilitation and Exercise

All patients with impaired exercise capacity must participate in a supervised 6-8 week pulmonary rehabilitation program 1, 2, 3. This is a strong recommendation based on high-quality evidence showing improvements in exercise capacity, reduced cough symptoms, enhanced quality of life, and decreased exacerbation frequency 1, 2.

• Encourage regular physical exercise (plus forced expiration technique/huff) to promote airway clearance 1
• Maintain regular exercise after formal rehabilitation completion 3

Bronchodilator Therapy

Offer a trial of long-acting bronchodilators (LABA, LAMA, or combination) for patients with significant breathlessness, particularly those with chronic airflow limitation 2:

• Administer before physiotherapy sessions and before inhaled antibiotics to improve drug deposition 2
• Discontinue if no symptomatic improvement after trial period 1, 2
• Perform reversibility testing to identify coexisting asthma, though not required to predict bronchodilator benefit 2

Management of Acute Exacerbations

Antibiotic Duration and Selection

Treat every exacerbation with 14 days of antibiotics 1, 2, 3. This duration is superior to shorter courses in reducing treatment failure 2.

• Obtain sputum culture before starting antibiotics whenever possible 2, 3
• Base antibiotic selection on most recent sputum culture and sensitivity results 1, 2, 3

Empiric Antibiotic Choices

When culture results are unavailable 1, 2:

• Streptococcus pneumoniae or Haemophilus influenzae (beta-lactamase negative): Amoxicillin 500 mg three times daily for 14 days
• Pseudomonas aeruginosa: Ciprofloxacin 500-750 mg twice daily for 14 days
• Consider intravenous antibiotics for severely unwell patients, resistant organisms, or failure to respond to oral therapy 2

Self-Management

Patients should keep appropriate antibiotics at home with a self-management plan for prompt self-initiation of therapy 2.

Eradication of New Pseudomonas aeruginosa

When P. aeruginosa is first isolated or re-emerges with clinical deterioration, offer eradication therapy 1, 2. P. aeruginosa infection is associated with three-fold increased mortality, seven-fold increased hospitalization risk, and one additional exacerbation per year 1, 2.

Eradication Protocol

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

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

Long-Term Antibiotic Therapy (≥3 Exacerbations Per Year)

Decision Algorithm

Step 1: Re-evaluate and optimize physiotherapy technique and airway clearance regimen 2

Step 2: If not already prescribed, initiate mucoactive therapy trial 2

Step 3: Implement long-term antibiotics based on microbiology 2:

For Chronic P. aeruginosa Infection

• First-line: Inhaled colistin (long-term) 2, 3
• Second-line: Inhaled gentamicin if colistin unsuitable 2
• Alternative: Long-term macrolide (azithromycin or erythromycin) if inhaled antibiotics contraindicated or not tolerated 2

For Non-Pseudomonas Infections

• Long-term macrolide therapy (e.g., azithromycin 250 mg three times weekly) after confirming absence of nontuberculous mycobacterial infection 2, 4

Monitoring Requirements

• Regular sputum cultures before and after implementing long-term antibiotics 3
• Monitor for drug toxicity, especially with macrolides and inhaled aminoglycosides 3
• Patients on long-term antibiotics require secondary-care follow-up 2

Anti-Inflammatory Treatments

Inhaled Corticosteroids

Do not routinely offer inhaled corticosteroids unless the patient has comorbid asthma or COPD 1, 2, 3. The diagnosis of bronchiectasis should not affect inhaled corticosteroid use in patients with these comorbidities 1.

Oral Corticosteroids

Do not offer long-term oral corticosteroids without other indications such as ABPA, chronic asthma, COPD, or inflammatory bowel disease 2, 3.

Exception: ABPA

For patients with ABPA, immunosuppression with corticosteroids (with or without antifungal agents) is the mainstay of treatment, using a tapering dose with monitoring of total serum IgE every 6-8 weeks as a disease activity marker 2.

Statins

Do not offer statins for treatment of bronchiectasis (strong recommendation) 1.

Immunizations

Mandatory vaccinations for all patients 2, 3:

• Annual influenza vaccination 2, 3
• 23-valent pneumococcal polysaccharide vaccine (single dose) 2
• Consider 13-valent pneumococcal conjugate vaccine if inadequate serologic response to polysaccharide vaccine 2
• Consider influenza vaccination for household contacts of patients with immune deficiency and bronchiectasis 2

Surgical Considerations

Surgery is not performed except in cases of localized disease with high exacerbation frequency despite optimization of all other management aspects 1, 2. Video-assisted thoracoscopic surgery (VATS) is preferred over open surgery to preserve lung function and reduce scarring 2.

Emergency surgery for massive hemoptysis in unstable patients carries 37% mortality 2.

Lung Transplantation Referral

Consider transplant referral for patients aged ≤65 years if 2:

• FEV1 <30% with significant clinical instability OR rapid progressive respiratory deterioration despite optimal medical management
• Additional factors warranting earlier referral: Massive hemoptysis, severe secondary pulmonary hypertension, ICU admissions, or respiratory failure

Common Pitfalls and Caveats

Critical Warning About Sedating Medications

Bronchiectasis patients require daily airway clearance for 10-30 minutes to expectorate sputum effectively 5. Any medication causing sedation (e.g., gabapentin) that prevents this fundamental therapy could worsen disease progression and increase exacerbation risk 5. If sedation contributes to inadequate airway clearance, the offending medication should be discontinued or reduced 5.

Do Not Extrapolate from Cystic Fibrosis

Treatment responses in non-cystic fibrosis bronchiectasis differ from cystic fibrosis bronchiectasis; specific guidance for non-CF bronchiectasis is necessary 1, 2.

Severity Assessment

Consider using the bronchiectasis severity index to guide management decisions 1.

CT Review for Physiotherapy

CT imaging should be reviewed to complement physiotherapy assessment and teach appropriate postural drainage positions 1.