What is the treatment approach for non-cystic fibrosis (CF) bronchiectasis in the United States (US)?

Treatment Approach for Non-Cystic Fibrosis Bronchiectasis in the United States

The treatment of non-cystic fibrosis bronchiectasis in the US follows a structured approach focused on optimizing airway clearance, treating underlying causes, managing infections, and using targeted antibiotic therapy based on pathogen identification. 1

Diagnostic Evaluation

• High-resolution CT (HRCT) scanning is the diagnostic procedure of choice to confirm bronchiectasis when suspected 1
• A comprehensive diagnostic evaluation for underlying disorders causing bronchiectasis should be performed, as identifying and treating these conditions may slow or halt disease progression 1
• Initial evaluation should include sputum cultures for bacteria, mycobacteria, and fungi to guide antimicrobial therapy 2

Core Treatment Components

Airway Clearance Techniques

• Chest physiotherapy should be used in patients with mucus hypersecretion and difficulty expectorating, with monitoring for symptom improvement 1
• Various techniques include:
  • Manual techniques: chest percussion, vibration, postural drainage
  • Mechanical interventions: mechanically assisted cough, airway oscillation devices 1, 3
• Although considered a mainstay in treatment, these techniques have modest effects on increasing sputum volume, and long-term effectiveness data is limited 1, 3

Pharmacological Management

Bronchodilators

• Bronchodilator therapy is recommended for patients with airflow obstruction and/or bronchial hyperreactivity 1
• Both β-agonists and antimuscarinic agents may be beneficial, particularly in patients with concurrent asthma or COPD 2

Mucoactive Agents

• Mucoactive treatments (≥3 months) may be offered to patients who have difficulty expectorating sputum and poor quality of life when standard airway clearance techniques have failed 1
• Hypertonic saline nebulization can help loosen tenacious secretions 2, 4
• Recombinant human DNase (rhDNase) should NOT be used in non-CF bronchiectasis patients 1

Antibiotic Therapy

For acute exacerbations:

• Antibiotics are a mainstay of treatment for exacerbations 1, 2
• Selection of agents should be guided by likely pathogens based on previous sputum cultures 1

For chronic infection management:

• Long-term antibiotic therapy should be considered for patients with ≥3 exacerbations per year 1, 2
• The approach differs based on pathogen:
  • For Pseudomonas aeruginosa infection:
    • Long-term inhaled antibiotics are recommended in CF but should NOT be used in non-CF bronchiectasis 1
    • Recent clinical trials with inhaled ciprofloxacin formulations show promise but are not yet standard practice 5
  • For non-Pseudomonas infection:
    • Long-term macrolide treatment is recommended 1, 4
    • Prior to starting macrolides, active nontuberculous mycobacterial (NTM) infection should be excluded as macrolide monotherapy can increase risk of resistance 1

Special Considerations

Management of Nontuberculous Mycobacteria (NTM)

• Careful evaluation of each respiratory NTM isolate is necessary in the context of the patient's overall status 1
• For patients with confirmed NTM pulmonary disease, treatment should involve collaboration with experts in NTM and bronchiectasis management 1
• When evaluating for NTM disease, patients should discontinue drugs that may compromise NTM culture (macrolides, fluoroquinolones, aminoglycosides, etc.) prior to sputum collection 1

Surgical Options

• Surgery to resect bronchiectatic lung should be limited to patients with localized disease who have not responded to medical therapy 1
• With proper patient selection, surgery is associated with excellent outcomes and low perioperative mortality rates 1

Monitoring and Follow-up

• Regular monitoring of sputum pathogens before and after implementation of long-term antibiotics is essential to:
  • Direct antibiotic choices
  • Monitor resistance patterns
  • Identify treatment-emergent organisms 1
• Drug toxicity monitoring is required, especially with macrolides and inhaled aminoglycosides 1

Common Pitfalls to Avoid

• Using recombinant human DNase (rhDNase) in non-CF bronchiectasis (may worsen outcomes) 1
• Using inhaled antibiotics in idiopathic non-CF bronchiectasis without careful patient selection 1, 5
• Starting macrolide therapy without first excluding active NTM infection 1
• Failing to identify and treat underlying conditions that may be contributing to bronchiectasis 1
• Neglecting airway clearance techniques as a fundamental component of management 1, 3