What are the current guideline recommendations for managing bronchiectasis in adults?

Management of Bronchiectasis in Adults

All adults with bronchiectasis should receive daily airway clearance techniques taught by a respiratory physiotherapist, combined with a 14-day antibiotic course for every exacerbation, and long-term inhaled antibiotics or macrolides for patients experiencing ≥3 exacerbations per year. 1, 2

Diagnostic Confirmation and Initial Workup

Imaging

• Perform thin-section CT scan to confirm bronchiectasis when clinically suspected, looking for bronchoarterial ratio >1, lack of bronchial tapering, or airway visibility within 1cm of pleural surface. 1
• Obtain baseline imaging during stable disease for optimal diagnostic accuracy and future comparison. 1

Minimum Laboratory Bundle

• Differential blood count to identify lymphopenia, neutropenia (suggesting immunodeficiency), or lymphocytosis (suggesting hematological malignancy). 1
• Serum immunoglobulins (IgG, IgA, IgM) because 2-8% have common variable immunodeficiency requiring immunoglobulin replacement therapy. 1
• Testing for allergic bronchopulmonary aspergillosis (ABPA) using total serum IgE, specific IgG and IgE to Aspergillus, or skin prick testing. 1, 3
• Sputum culture for bacteria and mycobacteria at every clinical visit to guide antibiotic selection. 1, 4

Additional Testing in Specific Circumstances

• Consider sweat chloride testing and CFTR gene analysis in young adults or those with upper-lobe predominant disease, nasal polyposis, chronic rhinosinusitis, recurrent pancreatitis, or male infertility. 1
• Obtain three sequential daily sputum cultures for mycobacteria in patients with radiological NTM features, weight loss, hemoptysis, or rapid deterioration. 1
• Perform bronchoscopy with bronchial aspiration in patients with localized disease or inability to expectorate, to exclude endobronchial lesions, foreign bodies, and improve NTM detection. 5

Airway Clearance: The Universal Foundation

First-Line Techniques

• Teach active cycle of breathing or oscillating positive expiratory pressure (PEP) devices as first-line options for all patients. 5
• Sessions should last 10-30 minutes, once or twice daily, continuing until two clear huffs or coughs are achieved. 5, 3, 4
• Incorporate forced-expiration (huff) maneuver with every session to mobilize mucus. 5

Positioning and Alternatives

• Use gravity-assisted positioning (modified postural drainage without head-down tilt) unless gastroesophageal reflux is present. 5
• When standard techniques fail, consider autogenic drainage, high-frequency chest-wall oscillation, or intrapulmonary percussive ventilation. 5

Monitoring

• Review technique within 3 months of initiation and conduct annual reassessment by a respiratory physiotherapist. 5
• During hospitalized exacerbations, provide daily physiotherapy visits until airway clearance is optimized. 5

Mucoactive Therapy

• Consider humidification with sterile water or normal saline to facilitate sputum clearance. 5, 3
• Trial carbocysteine for 6 months; continue only if clinical benefit is observed. 5
• Never use recombinant human DNase (dornase alfa) in non-CF bronchiectasis—it worsens clinical outcomes. 5, 3, 2

Management of Acute Exacerbations

Antibiotic Duration and Selection

• Treat every exacerbation with 14 days of antibiotics—this duration reduces treatment failure compared to shorter courses. 5, 3, 4, 2
• Obtain sputum culture before starting antibiotics whenever possible. 5, 3
• Base antibiotic selection on most recent sputum culture and sensitivity results. 5, 3, 4

Empiric Antibiotic Choices

• Amoxicillin 500mg three times daily for Streptococcus pneumoniae or beta-lactamase negative Haemophilus influenzae. 5
• Ciprofloxacin 500-750mg twice daily for Pseudomonas aeruginosa. 5
• Consider intravenous antibiotics for severely unwell patients, resistant organisms, or oral treatment failures. 3

Self-Management

• Patients should keep antibiotics at home with a self-management plan for prompt self-initiation during exacerbations. 5

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

For Chronic Pseudomonas aeruginosa Infection

• First-line: long-term inhaled colistin (strong recommendation). 5, 3, 2
• Second-line: inhaled gentamicin if colistin is unsuitable. 5
• Administer short-acting bronchodilator before inhaled antibiotics to prevent bronchospasm (occurs in 10-32% of patients). 5
• Perform supervised test dose with pre- and post-spirometry before initiating therapy. 5

P. aeruginosa infection carries a three-fold increase in mortality, seven-fold increase in hospitalization risk, and one additional exacerbation per year. 5

For Patients Without Pseudomonas aeruginosa

• First-line: macrolide therapy (azithromycin 250mg three times weekly or erythromycin). 5, 3, 2
• Exclude nontuberculous mycobacterial infection before starting macrolides because monotherapy promotes macrolide-resistant NTM. 5

Eradication of New Pseudomonas aeruginosa

• Offer eradication therapy when P. aeruginosa is first isolated or re-emerges with clinical deterioration. 5
• First-line: oral ciprofloxacin 500-750mg twice daily for 2 weeks. 5
• Second-line: 2 weeks IV antipseudomonal β-lactam ± aminoglycoside, followed by 3 months nebulized colistin, gentamicin, or tobramycin. 5
• Do not attempt eradication for pathogens other than P. aeruginosa. 5

Monitoring Requirements

• Comprehensive sputum analysis (bacteria, mycobacteria, fungi) before and after initiating chronic antibiotics to monitor resistance and detect emergent pathogens. 5
• Monitor for drug toxicity, especially ototoxicity with aminoglycosides and hepatic injury with macrolides. 5, 3

Pulmonary Rehabilitation

• Enroll all patients with impaired exercise capacity in supervised 6-8 week pulmonary rehabilitation (strong recommendation, high-quality evidence). 5, 3, 4, 2
• This intervention improves exercise capacity, reduces cough symptoms, enhances quality of life, and decreases exacerbation frequency. 5, 3, 4
• Maintain regular exercise after formal rehabilitation. 3

Bronchodilator Therapy

• Trial long-acting bronchodilators (LABA, LAMA, or combination) only in patients with significant breathlessness, particularly those with FEV₁/FVC <0.7. 5, 4
• Administer bronchodilators before physiotherapy sessions and before inhaled antibiotics to improve drug deposition. 5
• Discontinue if no symptomatic improvement after adequate trial. 5, 4, 2

Anti-Inflammatory Treatments

• Do not routinely prescribe inhaled corticosteroids unless comorbid asthma or COPD is present (strong recommendation). 5, 3, 4, 2
• Do not offer long-term oral corticosteroids without other indications such as ABPA, chronic asthma, COPD, or inflammatory bowel disease. 5, 4
• Do not use statins for bronchiectasis treatment (strong recommendation). 5

Exception: ABPA Management

• For ABPA, immunosuppression with corticosteroids ± antifungal agents is the mainstay, using tapering doses with monitoring of total serum IgE every 6-8 weeks. 5

Immunizations

• Offer annual influenza vaccination to all patients and household contacts of immunodeficient patients. 5, 3
• Offer 23-valent pneumococcal polysaccharide vaccine to all patients. 5, 3
• Consider 13-valent pneumococcal conjugate vaccine in patients with inadequate serologic response to polysaccharide vaccine. 5

Severity Assessment and Follow-Up

• Apply the Bronchiectasis Severity Index (BSI) to guide management decisions and prognosis. 5
• Obtain sputum culture at every clinical visit to guide antibiotic selection. 5
• Conduct at least annual comprehensive review to assess disease severity and optimize treatment. 5
• Patients with chronic P. aeruginosa infection, ≥3 exacerbations/year, or on long-term antibiotics should be followed in secondary-care services. 5

Surgical Considerations

• Surgery is reserved only for localized disease with high exacerbation frequency despite optimal medical management. 5, 4, 2
• Video-assisted thoracoscopic surgery (VATS) is preferred to preserve lung function and reduce scarring. 5
• Emergency surgery for massive hemoptysis carries mortality up to 37%. 5

Lung Transplantation Referral

• Refer patients ≤65 years for transplantation when FEV₁ <30% with significant clinical instability or rapid progressive respiratory decline despite optimal therapy. 5
• Consider earlier referral with massive hemoptysis, severe secondary pulmonary hypertension, ICU admissions, or respiratory failure. 5

Critical Pitfalls to Avoid

• Never extrapolate cystic fibrosis treatment data to non-CF bronchiectasis—treatment responses differ significantly. 5, 2
• Never use DNase in non-CF bronchiectasis—it causes clinical deterioration. 5, 3, 2
• Never start macrolides without excluding NTM infection—this promotes resistant organisms. 5
• Never use antibiotic courses shorter than 14 days for exacerbations—treatment failure rates increase. 5, 3, 4, 2