What is the management approach for bronchiectasis?

Management of Bronchiectasis

All patients with bronchiectasis and chronic productive cough must be taught airway clearance techniques by a trained respiratory physiotherapist, performing 10-30 minute sessions once or twice daily, and those with ≥3 exacerbations per year should receive long-term antibiotics—either inhaled antibiotics for chronic Pseudomonas aeruginosa infection or azithromycin 250 mg three times weekly for those without Pseudomonas. 1, 2, 3

Treatment Goals

The primary objectives are preventing exacerbations, reducing symptoms (cough, sputum, breathlessness), improving quality of life, and preventing disease progression including lung function decline and mortality. 4, 1, 2 Exacerbations are the major driver of mortality, lung function decline, and healthcare costs, with 50% of European patients experiencing ≥2 exacerbations annually and one-third requiring hospitalization. 1

Diagnostic Workup

• Imaging confirmation: High-resolution CT (HRCT) is the gold standard for confirming permanent bronchial dilatation. 1, 2, 5

• Initial laboratory evaluation: Obtain differential blood count, serum immunoglobulins (IgG, IgA, IgE, IgM), testing for allergic bronchopulmonary aspergillosis, and sputum culture for bacteria, mycobacteria, and fungi. 2, 5

• Pulmonary function: Perform prebronchodilator and postbronchodilator spirometry. 5

Non-Pharmacological Management (Mandatory Foundation)

Airway Clearance Techniques

• Strong recommendation: All patients with chronic productive cough or difficulty expectorating must be taught airway clearance techniques by a trained respiratory physiotherapist. 1, 2, 3

• Specific techniques: Active cycle of breathing, postural drainage, and manual or mechanical devices. 1, 2

• Dosing: Sessions lasting 10-30 minutes, performed once or twice daily. 1, 2, 6

Pulmonary Rehabilitation

• Strong recommendation: All patients with impaired exercise capacity should undergo pulmonary rehabilitation. 1, 2, 3

• Structure: 6-8 weeks of supervised exercise training. 6

• Benefits: Improved exercise capacity, reduced cough symptoms, enhanced quality of life, and decreased exacerbation frequency. 1, 2

Additional Non-Pharmacological Measures

• Nebulized saline: Use to loosen tenacious secretions. 5

• Regular exercise: Encourage ongoing physical activity beyond formal rehabilitation. 5

Pharmacological Management

Long-Term Antibiotic Therapy (For Frequent Exacerbators)

Indication: Patients with ≥3 exacerbations per year. 2, 3, 5

For Chronic Pseudomonas aeruginosa Infection:

• First-line: Long-term inhaled antibiotics (colistin or gentamicin). 1, 2, 5

• Rationale: P. aeruginosa infection confers 3-fold increased mortality risk, 7-fold increased hospitalization risk, and one additional exacerbation per year. 1

For Patients WITHOUT Pseudomonas aeruginosa:

• First-line: Azithromycin 250 mg three times weekly. 2, 6, 5

• Strong recommendation: Macrolides reduce exacerbations in this population. 1, 3

Acute Exacerbation Management

• Antibiotic duration: Treat all exacerbations with 14 days of antibiotics. 2, 6

• Selection: Base antibiotic choice on previous sputum culture results. 2, 6

• Severity consideration: Use intravenous antibiotics for severe exacerbations or treatment failures. 2

• Common pathogens: Haemophilus influenzae, Pseudomonas aeruginosa, Moraxella catarrhalis, Staphylococcus aureus, and Enterobacteriaceae. 1, 6

Mucoactive Treatments

• Consider for: Patients with difficulty expectorating sputum, poor quality of life, or failure of standard airway clearance techniques. 2

• Avoid: Do NOT use recombinant human DNase in non-CF bronchiectasis. 2

Bronchodilator Therapy

• Not routine: Do not routinely prescribe bronchodilators for all patients. 2, 3

• Consider for: Patients with significant breathlessness, chronic obstructive airflow limitation, or associated asthma. 2, 6

• Options: Long-acting β-agonists and antimuscarinic agents. 5

Anti-Inflammatory Treatments

• Inhaled corticosteroids: Do NOT routinely offer unless comorbid asthma or COPD is present. 2, 3, 5

• Oral corticosteroids: Do NOT offer long-term oral corticosteroids. 2

Monitoring and Follow-Up

• Sputum surveillance: Monitor sputum pathogens regularly, especially when using long-term antibiotics. 2

• Drug toxicity monitoring: Monitor for toxicity with macrolides and inhaled aminoglycosides. 2

• Physiotherapy assessment: Annual assessment by respiratory physiotherapist to optimize airway clearance regimen. 2

• Pulse oximetry: Screen for respiratory failure. 6

• Frequency: Minimum annual assessments, with more frequent monitoring in severe disease. 6

Surgical Intervention

• Not routine: Do NOT routinely recommend surgery for most patients. 2, 6, 3

• Consider only for: Patients with localized disease and high exacerbation frequency despite optimization of all other management aspects. 2, 6

• Lung transplant: Reserve for patients with severely impaired pulmonary function, frequent exacerbations, or both. 5

Vaccination

• Strongly recommended: Pneumococcal and influenza vaccines are crucial for preventing infections and complications. 7

Critical Pitfalls to Avoid

• Underutilization of physiotherapy: Airway clearance techniques and pulmonary rehabilitation have strong evidence but are frequently underused. 1

• Failure to aggressively treat P. aeruginosa: Missing or inadequately treating Pseudomonas infection dramatically worsens outcomes. 1

• Inadequate etiological workup: Missing treatable causes like immunodeficiency or allergic bronchopulmonary aspergillosis. 1

• Inappropriate use of inhaled corticosteroids: Do not prescribe routinely without comorbid asthma or COPD. 2, 3

• Using recombinant human DNase: This is contraindicated in non-CF bronchiectasis. 2