Initial Management and Treatment of Bronchiectasis

Core Management Strategy

All patients with bronchiectasis should receive airway clearance techniques taught by a trained respiratory physiotherapist, performing sessions for 10-30 minutes once or twice daily, combined with regular physical exercise and pulmonary rehabilitation for those with impaired exercise capacity. 1, 2

Non-Pharmacological Management (First-Line for All Patients)

Airway Clearance Techniques

Teach all patients with chronic productive cough or difficulty expectorating sputum an airway clearance technique performed in the sitting position, including active cycle of breathing, autogenic drainage, positive expiratory pressure devices (Flutter, Acapella), or high-frequency chest wall oscillation. 1, 2
Sessions should last minimum 10 minutes up to maximum 30 minutes, continuing until two clear huffs/coughs are completed or fatigue develops. 1
Frequency should be once or twice daily during stable disease, with increased frequency during exacerbations. 1, 2
Reassess by respiratory physiotherapist within 3 months of initial teaching and annually thereafter as part of clinical review. 1

Pulmonary Rehabilitation and Exercise

All patients with impaired exercise capacity should participate in pulmonary rehabilitation programs consisting of 6-8 weeks of supervised exercise training. 1, 2, 3
Encourage regular physical exercise plus forced expiration technique/huff to promote airway clearance even in those without formal rehabilitation. 1

Initial Diagnostic Workup

Essential Testing at Diagnosis

Differential blood count to assess for eosinophilia or immunodeficiency patterns. 4, 3
Serum immunoglobulins (IgG, IgA, IgE, IgM) to identify immunodeficiency syndromes. 4, 3, 5
Testing for allergic bronchopulmonary aspergillosis (ABPA) with total IgE and Aspergillus-specific IgE. 4, 3
Sputum culture and sensitivity for bacteria, mycobacteria, and fungi to guide antibiotic therapy. 4, 3, 5
Prebronchodilator and postbronchodilator spirometry to assess airflow obstruction and reversibility. 5

Pharmacological Management

Mucoactive Treatments

Consider humidification with sterile water or normal saline to facilitate airway clearance. 1, 2
Consider trial of mucoactive treatment (e.g., carbocysteine for 6 months) in patients with difficulty expectorating sputum and poor quality of life where standard airway clearance techniques have failed. 1, 2
Perform airway reactivity challenge test when first administering inhaled mucoactive treatment. 1
Consider pre-treatment with bronchodilator prior to inhaled/nebulized mucoactive treatments, especially in patients with asthma, bronchial hyperreactivity, or severe airflow obstruction (FEV1 <1 liter). 1
Do NOT routinely use recombinant human DNase (dornase alfa) in adults with non-CF bronchiectasis. 1, 2

Bronchodilator Therapy

Do NOT routinely offer long-acting bronchodilators to all patients with bronchiectasis. 1, 3
Consider bronchodilators for patients with significant breathlessness on an individual basis, with appropriate inhalation device selection and technique training. 1, 3
Use bronchodilators before physiotherapy sessions, inhaled mucoactive drugs, and inhaled antibiotics to increase tolerability and optimize pulmonary deposition. 1, 4
Continue bronchodilators in patients with comorbid asthma or COPD according to standard guidelines for those conditions. 1
Discontinue bronchodilators if no symptom reduction is achieved. 1

Anti-Inflammatory Treatments

Do NOT routinely offer inhaled corticosteroids unless comorbid asthma, COPD, ABPA, or inflammatory bowel disease is present. 1, 2, 3
Do NOT offer long-term oral corticosteroids without other indications (ABPA, chronic asthma, COPD, inflammatory bowel disease). 1, 2
Do NOT routinely offer PDE4 inhibitors, methylxanthines, leukotriene receptor antagonists, CXCR2 antagonists, neutrophil elastase inhibitors, or statins for bronchiectasis treatment. 1, 4

Management of Acute Exacerbations

Antibiotic Treatment for Exacerbations

Treat all exacerbations with 14 days of antibiotics, selected based on previous sputum culture results. 1, 2, 4, 3
Obtain sputum for culture and sensitivity before starting antibiotics whenever possible, then start empirical therapy while awaiting results. 1, 2
Modify antibiotics based on culture results if no clinical improvement occurs. 1

First-Line Antibiotic Choices by Pathogen

Streptococcus pneumoniae: Amoxicillin 500mg three times daily for 14 days. 2, 4
Haemophilus influenzae (beta-lactamase negative): Amoxicillin 500mg three times daily for 14 days. 2, 4
Pseudomonas aeruginosa: Ciprofloxacin 500-750mg twice daily for 14 days (always 14 days for P. aeruginosa). 1, 2, 4
Consider intravenous antibiotics for patients who are particularly unwell, have resistant organisms, or have failed oral therapy. 4

Patient Self-Management

Provide suitable patients with antibiotics to keep at home for prompt treatment of exacerbations. 1
Consider a patient self-management plan to facilitate early recognition and treatment of exacerbations. 1

Long-Term Antibiotic Therapy (For Frequent Exacerbators)

Indications

Consider long-term antibiotics only for patients with ≥3 exacerbations per year after optimizing airway clearance and treating modifiable underlying causes. 1, 2, 3

For Chronic Pseudomonas aeruginosa Infection

First-line: Long-term inhaled antibiotics (colistin, gentamicin, or tobramycin). 1, 2, 3
Consider adding or substituting macrolides (azithromycin or erythromycin) if high exacerbation frequency persists despite inhaled antibiotics. 1

For Patients WITHOUT Pseudomonas aeruginosa

First-line: Long-term macrolides (azithromycin or erythromycin). 1, 2, 3
Alternative: Long-term oral antibiotic (choice based on susceptibility and tolerance) if macrolides are contraindicated, not tolerated, or ineffective. 1
Alternative: Long-term inhaled antibiotic if oral prophylaxis is contraindicated, not tolerated, or ineffective. 1

Pseudomonas Aeruginosa Eradication

Offer eradication treatment for new growth of P. aeruginosa (first isolation or regrowth with intermittently positive cultures) associated with clinical deterioration. 1
First-line eradication: Ciprofloxacin 500-750mg twice daily for 2 weeks. 1
Second-line eradication: IV antipseudomonal beta-lactam ± IV aminoglycoside for 2 weeks, followed by 3 months of nebulized colistin, gentamicin, or tobramycin. 1
Discuss risks and benefits of eradication treatment versus clinical observation with patients. 1
Send sputum for culture immediately before and at each attendance following eradication antibiotics to determine outcome. 1

Immunizations

Offer annual influenza immunization to all patients with bronchiectasis. 1, 2
Offer polysaccharide pneumococcal vaccination (23-valent) to all patients with bronchiectasis. 1, 2
Consider 13-valent protein conjugate pneumococcal vaccine in patients without appropriate serological response to standard polysaccharide vaccine. 1
Consider influenza vaccination in household contacts of patients with immune deficiency and bronchiectasis to reduce secondary transmission risk. 1

Surgical Intervention (Highly Selected Cases Only)

Do NOT offer surgical treatment except for patients with localized disease and high exacerbation frequency despite optimization of all other management aspects. 1, 4, 3
Video-assisted thoracoscopic surgery (VATS) is preferred over open surgery when surgery is indicated, with shorter hospital stay, fewer complications (17.5% vs 23.7%), and less pain. 1
Pooled surgical mortality is 1.4% and post-operative morbidity is 16.2% in observational studies. 1, 4
Emergency surgery for massive hemoptysis carries mortality up to 37% in unstable patients. 1, 2

Lung Transplantation Referral

Consider transplant referral for patients aged ≤65 years if FEV1 <30% with significant clinical instability or rapid progressive respiratory deterioration despite optimal medical management. 1, 2
Consider earlier referral with additional factors: massive hemoptysis, severe secondary pulmonary hypertension, ICU admissions, or respiratory failure (particularly requiring NIV). 1, 2
Discuss with transplant center prior to formal referral and optimize comorbidities (osteoporosis) and physical condition through pulmonary rehabilitation. 1

Common Pitfalls and Caveats

Do NOT extrapolate treatments from cystic fibrosis bronchiectasis, as treatment responses differ significantly. 2
Recognize that P. aeruginosa infection is associated with three-fold increase in mortality risk, almost seven-fold increase in hospital admission risk, and one additional exacerbation per patient per year. 2, 4
Monitor for drug toxicity with long-term macrolides (hearing loss, QT prolongation, drug interactions) and inhaled aminoglycosides (renal function, hearing). 3
Regular sputum monitoring is essential when using long-term antibiotics to detect emerging resistance. 3
During exacerbations, consider intermittent positive pressure breathing or non-invasive ventilation to reduce work of breathing in fatigued/breathless patients, allowing longer treatment sessions and postural drainage positions. 1, 2

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