What are the management strategies for bronchiectasis?

Bronchiectasis Management

Definition and Core Pathophysiology

Bronchiectasis is a chronic respiratory disease characterized by permanent bronchial dilatation, chronic cough with sputum production, and recurrent infections, driven by a self-perpetuating cycle of chronic bacterial infection, neutrophilic inflammation, impaired mucociliary clearance, and progressive structural lung damage. 1

• The disease is maintained by chronic bacterial colonization with Haemophilus influenzae, Pseudomonas aeruginosa, Moraxella catarrhalis, Staphylococcus aureus, and Enterobacteriaceae 1
• Neutrophilic inflammation causes elastin degradation and accelerated lung function decline 1
• Quality of life impairment equals that of severe COPD and idiopathic pulmonary fibrosis 1
• 50% of European patients experience ≥2 exacerbations annually, with one-third requiring hospitalization 1
• P. aeruginosa infection confers 3-fold increased mortality risk, 7-fold increased hospitalization risk, and one additional exacerbation per year 1, 2

Diagnostic Confirmation

• High-resolution CT (HRCT) is the gold standard for confirming permanent bronchial dilatation 1, 3
• Initial workup must include differential blood count, serum immunoglobulins (IgG, IgA, IgE, IgM), testing for allergic bronchopulmonary aspergillosis, and sputum culture for bacteria, mycobacteria, and fungi 3, 4
• Prebronchodilator and postbronchodilator spirometry should be performed 4

Treatment Goals

The primary objectives are preventing exacerbations, reducing symptom burden, improving quality of life, and preventing disease progression (lung function decline and mortality). 1

Non-Pharmacological Management (Foundation of 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. 5, 3, 2

• Techniques include active cycle of breathing, postural drainage, and manual or mechanical devices 3
• Sessions should last 10-30 minutes, performed once or twice daily 3, 2
• This is a cornerstone intervention that should never be omitted 1

Pulmonary Rehabilitation

Patients with impaired exercise capacity should participate in pulmonary rehabilitation programs—this is a strong recommendation with high-quality evidence. 5, 3

• Benefits include improved exercise capacity, reduced cough symptoms, enhanced quality of life, and decreased exacerbation frequency 3, 2
• Regular exercise should be maintained long-term 5

Pharmacological Management

Acute Exacerbation Treatment

Treat exacerbations with 14 days of antibiotics, selected based on previous sputum culture results. 3, 2

• Obtain sputum cultures before starting antibiotics whenever possible 2
• Common pathogens and recommended antibiotics: 2
  • Streptococcus pneumoniae: Amoxicillin 500mg three times daily (14 days)
  • Haemophilus influenzae (beta-lactamase negative): Amoxicillin 500mg three times daily (14 days)
  • Pseudomonas aeruginosa: Ciprofloxacin 500-750mg twice daily (14 days)
• Consider intravenous antibiotics for severe exacerbations, treatment failures, or resistant organisms 3, 2

Long-Term Antibiotic Therapy (For Frequent Exacerbators)

Consider long-term antibiotics only for patients with ≥3 exacerbations per year, and only after optimizing airway clearance and treating modifiable underlying causes. 5, 3, 2

For Patients WITH Chronic Pseudomonas aeruginosa Infection:

First-line: Long-term inhaled antibiotics (colistin or gentamicin). 1, 3, 4

• This is the preferred approach given the dramatic impact of P. aeruginosa on mortality and hospitalization 1, 2
• Macrolides (azithromycin, erythromycin) can be added to or used in place of inhaled antibiotics if high exacerbation frequency persists despite inhaled antibiotic therapy 5

For Patients WITHOUT Pseudomonas aeruginosa Infection:

First-line: Macrolides (azithromycin, erythromycin). 5, 3, 4

• This recommendation is based on moderate quality evidence 5
• If macrolides are contraindicated, not tolerated, or ineffective, consider long-term oral antibiotics based on antibiotic susceptibility and patient tolerance 5
• Inhaled antibiotics can be used if oral prophylaxis is contraindicated, not tolerated, or ineffective 5

Mucoactive Treatments

• Consider long-term mucoactive treatment (≥3 months) for patients with difficulty expectorating sputum and poor quality of life where standard airway clearance techniques have failed 5, 3
• Nebulization with sterile water or normal saline can facilitate airway clearance 2
• Do NOT use recombinant human DNase (dornase alfa) in non-CF bronchiectasis—this is a strong recommendation with moderate quality evidence. 5, 3, 2

Bronchodilator Therapy

Do not routinely offer long-acting bronchodilators for all patients with bronchiectasis. 5, 3

• Consider long-acting bronchodilators only for patients with significant breathlessness on an individual basis 5, 3
• Use bronchodilators before physiotherapy, inhaled mucoactive drugs, and inhaled antibiotics to increase tolerability and optimize pulmonary deposition 5
• The diagnosis of bronchiectasis should not affect the use of long-acting bronchodilators in patients with comorbid asthma or COPD 5

Anti-Inflammatory Treatments

Do not routinely offer inhaled corticosteroids unless comorbid asthma or COPD is present. 3, 2

• Do not offer long-term oral corticosteroids without other indications such as ABPA, chronic asthma, COPD, or inflammatory bowel disease 2
• For allergic bronchopulmonary aspergillosis (ABPA), immunosuppression with corticosteroids, with or without antifungal agents, is the mainstay of treatment 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 management. 5, 3, 2

• Video-assisted thoracoscopic surgery (VATS) is preferred over open surgery to better preserve lung function and reduce scarring 2
• Emergency surgery in unstable patients with massive hemoptysis carries mortality rates reaching 37% 2
• Lung transplant should be considered for patients aged ≤65 years if FEV1 is <30% with significant clinical instability or rapid progressive respiratory deterioration despite optimal medical management 2

Immunizations

Offer annual influenza immunization to all patients with bronchiectasis. 2

• Pneumococcal vaccination should be offered to all patients 2
• Consider influenza vaccination in household contacts of patients with immune deficiency and bronchiectasis 2

Monitoring and Follow-Up

• Regular monitoring of sputum pathogens is essential, especially when using long-term antibiotics 3
• Monitor for drug toxicity, particularly with macrolides and inhaled aminoglycosides 3
• Annual assessment by respiratory physiotherapist to optimize airway clearance regimen 3

Critical Clinical Pitfalls

The most common errors in bronchiectasis management are underutilization of airway clearance techniques and pulmonary rehabilitation despite strong evidence, and failure to identify and aggressively treat P. aeruginosa infection given its dramatic impact on outcomes. 1

• Inadequate etiological workup can miss treatable causes like immunodeficiency or ABPA 1
• Do not extrapolate treatments from cystic fibrosis bronchiectasis, as treatment responses are different 2
• Exacerbations drive mortality, lung function decline, and healthcare costs—aggressive prevention is paramount 1