What is the management approach for chronic bronchiectasis?

Management of Chronic Bronchiectasis

All patients with chronic bronchiectasis must receive airway clearance techniques taught by a trained respiratory physiotherapist, and those with ≥3 exacerbations per year should receive long-term antibiotics—either inhaled antibiotics for Pseudomonas aeruginosa infection or macrolides for non-Pseudomonas disease—after optimizing airway clearance. 1, 2, 3

Primary Treatment Goals

The management strategy targets four interconnected disease mechanisms to prevent exacerbations, reduce symptoms, improve quality of life, and halt disease progression (lung function decline and mortality). 4, 1

• Chronic bacterial infection with Haemophilus influenzae, Pseudomonas aeruginosa, Moraxella catarrhalis, Staphylococcus aureus, and Enterobacteriaceae drives the disease cycle. 4, 1
• Neutrophilic inflammation causes elastin degradation in airways, leading to rapid lung function decline and increased exacerbation frequency. 4, 1
• Impaired mucociliary clearance results from structural damage, airway dehydration, and excessive mucus viscosity, with >70% of patients expectorating sputum daily. 1
• Progressive structural lung damage perpetuates the vicious cycle of disease. 4

Diagnostic Confirmation and Initial Workup

• High-resolution CT (HRCT) without contrast is the gold standard for confirming permanent bronchial dilatation. 1, 5, 3
• All patients must undergo comprehensive etiological workup including differential blood count, serum immunoglobulins (IgG, IgA, IgE, IgM), testing for allergic bronchopulmonary aspergillosis, and sputum culture for bacteria, mycobacteria, and fungi. 2, 5
• Prebronchodilator and postbronchodilator spirometry should be performed. 5

Non-Pharmacological Management (Foundational Therapy)

Airway Clearance Techniques

All patients with chronic productive cough or difficulty expectorating sputum must be taught airway clearance techniques by a trained respiratory physiotherapist—this is a strong recommendation based on high-quality evidence. 1, 2, 3

• Techniques include active cycle of breathing, postural drainage, and manual or mechanical devices. 2
• Sessions should last 10-30 minutes, performed once or twice daily. 2
• Annual reassessment by a respiratory physiotherapist is essential to optimize the airway clearance regimen. 2

Pulmonary Rehabilitation

Patients with impaired exercise capacity should participate in pulmonary rehabilitation programs—this is a strong recommendation with high-quality evidence. 1, 2, 3

• Benefits include improved exercise capacity, reduced cough symptoms, enhanced quality of life, and decreased exacerbation frequency. 2

Pharmacological Management

Acute Exacerbation Treatment

• Treat all exacerbations with 14 days of antibiotics to reduce treatment failure risk and improve outcomes. 1, 2
• Select antibiotics based on previous sputum culture results. 2
• Obtain sputum cultures before starting antibiotics whenever possible. 2

Long-Term Antibiotic Therapy

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

For Chronic Pseudomonas aeruginosa Infection:

• First-line treatment is long-term inhaled antibiotics (colistin or gentamicin)—this is a strong recommendation. 1, 2, 3
• P. aeruginosa infection confers a 3-fold increased mortality risk, 7-fold increased hospitalization risk, and one additional exacerbation per year. 1, 6

For Patients Without Pseudomonas aeruginosa Infection:

• First-line treatment is macrolides (azithromycin or erythromycin). 1, 2, 3

Bronchodilators and Inhaled Corticosteroids

• Do not routinely offer long-acting bronchodilators for all patients with bronchiectasis. 2
• Do not routinely offer inhaled corticosteroids unless comorbid asthma or COPD is present. 2, 5
• Inhaled bronchodilators (β-agonists and antimuscarinic agents) are indicated only when asthma or COPD coexists. 5

Mucoactive Therapy

• Nebulization of saline may be used to loosen tenacious secretions. 5

Immunizations

• All patients must receive annual influenza immunization. 1, 2
• Pneumococcal vaccination should be offered to all patients. 2

Surgical Management

• Do not offer surgical treatments except for patients with localized disease and high exacerbation frequency despite optimization of all other management aspects. 1, 2
• Video-assisted thoracoscopic surgery (VATS) is preferred over open surgery to better preserve lung function and reduce scarring. 2

Lung Transplantation

• Consider transplant referral for patients aged ≤65 years if FEV1 is <30% with significant clinical instability or rapid progressive respiratory deterioration despite optimal medical management. 1, 2

Monitoring and Follow-Up

• Regular monitoring of sputum pathogens is essential, especially when using long-term antibiotics. 2
• Monitor for drug toxicity, particularly with macrolides and inhaled aminoglycosides. 2
• Annual assessment by respiratory physiotherapist to optimize airway clearance regimen. 2

Critical Clinical Pitfalls to Avoid

• The most common error is underutilization of airway clearance techniques and pulmonary rehabilitation despite strong evidence for their benefit. 1, 6
• Failure to identify and treat P. aeruginosa infection aggressively given its dramatic impact on outcomes (3-fold mortality increase, 7-fold hospitalization increase). 1, 6
• Inadequate etiological workup missing treatable causes like immunodeficiency or allergic bronchopulmonary aspergillosis. 1, 6
• Never use recombinant human DNase (dornase alfa) in non-CF bronchiectasis, as it may worsen outcomes. 1
• Do not extrapolate treatments from cystic fibrosis bronchiectasis, as treatment responses differ. 1

Prognostic Considerations

• Mortality is higher in patients with frequent and severe exacerbations, Pseudomonas aeruginosa infection, and comorbidities such as COPD. 1, 6, 5
• Breathlessness is one of the strongest predictors of mortality. 1
• Up to 30% mortality at 1-year follow-up after suffering an exacerbation, particularly with comorbid COPD. 1
• 50% of European patients experience ≥2 exacerbations annually, with one-third requiring hospitalization. 4, 1