What is the recommended management of bronchiectasis in an adult patient without specific comorbidities?

Management of Bronchiectasis in Adults

All adults with bronchiectasis should receive daily airway clearance techniques taught by a trained respiratory physiotherapist as the cornerstone of therapy, combined with pulmonary rehabilitation for those with impaired exercise capacity, and 14-day antibiotic courses for every exacerbation. 1

Initial Diagnostic Workup

Obtain a minimum bundle of etiological tests at diagnosis: 1

• Differential blood count 1
• Serum immunoglobulins (IgG, IgA, IgM) 1
• Testing for allergic bronchopulmonary aspergillosis (ABPA) 1
• Sputum culture for bacteria and mycobacteria at every clinical visit 1, 2
• High-resolution CT scan to confirm permanent bronchial dilatation 3

Additional tests should be pursued in patients with severe or rapidly progressive disease, or when specific clinical features suggest particular etiologies. 1

Airway Clearance Techniques (Foundation of All Management)

Every patient with chronic productive cough or difficulty expectorating sputum must be taught airway clearance techniques by a trained respiratory physiotherapist. 1, 2

• Perform 10-30 minute sessions once or twice daily until two clear huffs or coughs are achieved 2
• First-line techniques: active cycle of breathing or oscillating positive-expiratory-pressure (PEP) devices 2
• Incorporate the forced-expiration (huff) maneuver with every airway clearance method 2
• Use gravity-assisted positioning (modified postural drainage without head-down tilt) unless contraindicated by gastroesophageal reflux 2
• Review technique within 3 months of initiation and conduct annual reassessment 2
• During hospitalizations, provide daily physiotherapy visits until airway clearance is optimized 2

Alternative methods (autogenic drainage, high-frequency chest-wall oscillation, intrapulmonary percussive ventilation) should be used when standard techniques are ineffective or not tolerated. 2

Pulmonary Rehabilitation and Exercise

Patients with impaired exercise capacity must participate in a supervised 6-8 week pulmonary rehabilitation program. 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, 3

• Encourage regular physical exercise combined with the forced-expiration technique to further promote airway clearance 2
• Benefits are maintained long-term with continued exercise 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. 1, 4, 2, 3

Antibiotic Selection Strategy:

• Select antibiotics based on the most recent sputum culture and sensitivity results obtained before therapy whenever possible 1, 4, 2
• Obtain sputum for culture before starting antibiotics at every exacerbation 2
• Patients should keep a supply of appropriate antibiotics at home with a self-management plan for prompt self-initiation 2

Empiric Therapy When Cultures Are Unavailable:

• Amoxicillin 500mg three times daily for Streptococcus pneumoniae or Haemophilus influenzae (beta-lactamase negative) 2
• Ciprofloxacin 500-750mg twice daily for Pseudomonas aeruginosa 4, 2
• Amoxicillin-clavulanate or trimethoprim-sulfamethoxazole for E. coli based on susceptibility patterns 4

Route of Administration:

• Oral antibiotics are first-line for most exacerbations 4
• Switch to intravenous antibiotics for severe exacerbations, treatment failures after oral therapy, or if the patient is acutely deteriorating 4, 2
• Extend treatment beyond 14 days only if the patient has not returned to baseline clinical state by day 14; re-evaluate clinically and obtain new sputum culture at this point 4

Eradication of New Pseudomonas aeruginosa Isolation

Offer eradication therapy when P. aeruginosa is first isolated or re-emerges with clinical deterioration—this pathogen is associated with a three-fold increase in mortality, seven-fold increase in hospitalization risk, and one additional exacerbation per year. 1, 2, 3

Eradication Protocol:

• First-line: oral ciprofloxacin 500-750mg 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 attempt eradication for pathogens other than P. aeruginosa. 1

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

Consider long-term prophylactic antibiotics only after optimizing airway clearance techniques and treating modifiable underlying causes. 1, 2

For Chronic P. aeruginosa Infection:

• First-line: long-term inhaled antibiotics (colistin or gentamicin) 1, 2, 3
• Second-line: macrolides (azithromycin or erythromycin) if inhaled antibiotics are contraindicated, not tolerated, or not feasible 1
• Consider adding macrolides to inhaled antibiotics for patients with high exacerbation frequency despite inhaled antibiotic therapy 1

For Patients Without P. aeruginosa Infection:

• First-line: long-term macrolides (azithromycin 250mg three times weekly or erythromycin) after confirming absence of nontuberculous mycobacterial infection 1, 2, 3
• Second-line: oral antibiotics (choice based on antibiotic susceptibility and patient tolerance) if macrolides are contraindicated, not tolerated, or ineffective 1
• Third-line: inhaled antibiotics if oral antibiotic prophylaxis is contraindicated, not tolerated, or ineffective 1

Monitoring on Long-Term Antibiotics:

• Monitor for drug toxicity, particularly with macrolides and inhaled aminoglycosides 3
• Monitor sputum pathogens regularly when using long-term antibiotics 3

Mucoactive Therapy

Consider long-term mucoactive treatment (≥3 months) for patients with difficulty expectorating sputum, poor quality of life, or failure of standard airway clearance techniques. 1, 2, 3

• Humidification with sterile water or normal saline may facilitate sputum clearance 2
• A 6-month trial of carbocysteine is reasonable; continue only if clinical benefit is observed 2
• Perform airway-reactivity testing before initiating inhaled mucoactive agents 2

Critical Contraindication:

Recombinant human DNase (dornase alfa) must not be used in non-cystic fibrosis bronchiectasis—this is a strong recommendation based on moderate-quality evidence showing it worsens outcomes. 1, 2, 3

Bronchodilator Therapy

Do not routinely offer long-acting bronchodilators for all patients with bronchiectasis. 1, 3

• Offer a trial of long-acting bronchodilators (LABA, LAMA, or combination) for patients with significant breathlessness on an individual basis 1, 2
• Discontinue bronchodilator therapy if no symptomatic improvement is observed after the trial period 1, 2
• Use bronchodilators before physiotherapy sessions and before inhaled antibiotics to improve pulmonary drug deposition and increase tolerability 1
• The diagnosis of bronchiectasis should not affect the use of long-acting bronchodilators in patients with comorbid asthma or COPD 1

Anti-Inflammatory Treatments

Do not routinely offer inhaled corticosteroids unless the patient has comorbid asthma or COPD. 1, 2, 3

• The diagnosis of bronchiectasis should not affect the use of inhaled corticosteroids in patients with comorbid asthma or COPD 1
• Do not offer statins for the treatment of bronchiectasis—this is a strong recommendation 1
• Do not offer long-term oral corticosteroids without other indications such as ABPA, chronic asthma, COPD, or inflammatory bowel disease 2

Special Consideration for ABPA:

For patients with allergic bronchopulmonary aspergillosis, 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 marker of disease activity. 2

Immunizations

Offer annual influenza vaccination to all patients with bronchiectasis. 2

Offer pneumococcal vaccination to all patients:

• Single dose of 23-valent pneumococcal polysaccharide vaccine 2
• Consider 13-valent pneumococcal conjugate vaccine in patients who do not achieve adequate serologic response to the polysaccharide vaccine 2

Surgical Intervention

Do not offer surgical treatments except for patients with localized disease and high exacerbation frequency despite optimization of all other aspects of bronchiectasis management. 1, 2, 3

• Video-assisted thoracoscopic surgery (VATS) is often preferred to better preserve lung function and reduce scarring compared to open surgery 2
• Emergency surgery in unstable patients with massive hemoptysis is associated with higher morbidity and mortality reaching 37% 2

Lung Transplant Referral

Refer patients ≤65 years for lung transplantation when:

• FEV₁ <30% with significant clinical instability or rapid progressive respiratory decline despite optimal medical therapy 2
• Additional factors: massive hemoptysis, severe secondary pulmonary hypertension, ICU admissions, or respiratory failure 2

Monitoring and Follow-Up

• Obtain sputum for culture at every clinical visit to guide antibiotic selection 2, 3
• Conduct at least an annual comprehensive review to assess disease severity and optimize all treatment components 2
• Patients with chronic P. aeruginosa infection, ≥3 exacerbations per year, or on long-term antibiotics should be followed in secondary-care services 2
• Apply the Bronchiectasis Severity Index to guide management decisions 2
• Review CT imaging alongside physiotherapy assessment to tailor postural drainage positions 2

Common Pitfalls to Avoid

Do not extrapolate cystic fibrosis treatment data to non-CF bronchiectasis—treatment responses are different and specific non-CF recommendations are required. 2, 5

Do not use shorter antibiotic courses (<14 days) for exacerbations except for mild exacerbations in patients with mild disease or rapid return to baseline state, as evidence supporting shorter courses is lacking. 4

Ensure patient education and cooperation with healthcare providers to implement treatment plans, as this is key to successful disease management. 6