How to manage bronchiectasis according to American Thoracic Society (ATS) and British Thoracic Society (BTS) guidelines?

Management of Bronchiectasis According to ATS/BTS Guidelines

All adults with bronchiectasis should be taught airway clearance techniques by a trained respiratory physiotherapist, performing 10-30 minute sessions once or twice daily, as this forms the cornerstone of non-pharmacological management. 1

Initial Assessment and Diagnosis

• Confirm diagnosis with thin-section CT chest showing irreversibly damaged and dilated bronchi 1
• Obtain baseline sputum for culture and sensitivity testing to identify chronic pathogens, particularly Pseudomonas aeruginosa, which increases mortality risk three-fold 1, 2
• Investigate underlying causes: immunoglobulin levels (IgG, IgA, IgE, IgM), ABPA screening (total IgE, Aspergillus specific IgE), autoimmune markers if clinically indicated, and consider primary ciliary dyskinesia testing 1, 2
• Perform baseline spirometry (pre- and post-bronchodilator) and pulse oximetry 1, 2

Airway Clearance (Foundation of Management)

Active cycle of breathing technique in the sitting position should be taught as the first-line airway clearance method. 1

• Sessions should last 10-30 minutes, performed once or twice daily, continuing until two clear huffs/coughs are completed 1
• Alternative techniques include autogenic drainage, positive expiratory pressure devices, or high-frequency chest wall oscillation if initial technique is ineffective or unacceptable 1
• Review technique within 3 months of initial assessment, then annually, or whenever clinical deterioration occurs 1, 2
• Consider pre-treatment with bronchodilators before airway clearance sessions, especially in patients with airflow obstruction (FEV1 <1 liter) 1

Mucoactive Therapy

• Consider humidification with sterile water or normal saline to facilitate airway clearance 1
• Trial carbocysteine for 6 months in patients with difficulty expectorating; continue only if ongoing clinical benefit 1
• Do not use recombinant human DNase (dornase alfa) in non-CF bronchiectasis 1
• Perform airway reactivity challenge test before starting any inhaled mucoactive treatment 1

Management of Acute Exacerbations

Treat all exacerbations with 14 days of antibiotics, selecting based on previous sputum culture results; always use 14 days for Pseudomonas aeruginosa infections. 1

• Obtain sputum for culture before starting antibiotics whenever possible, but do not delay treatment 1
• Start empirical antibiotics while awaiting culture results, then modify based on sensitivity testing if no clinical improvement 1
• Common pathogens requiring coverage: Haemophilus influenzae, Streptococcus pneumoniae, Moraxella catarrhalis, Staphylococcus aureus, and Pseudomonas aeruginosa 1
• Patients should have antibiotics at home for prompt self-initiation of treatment when exacerbation symptoms develop 1

Long-Term Antibiotic Prophylaxis

For patients with ≥3 exacerbations per year despite optimized airway clearance, consider long-term antibiotic therapy. 1, 2

For Chronic Pseudomonas aeruginosa Infection:

• First-line: Long-term inhaled antibiotics (colistin, gentamicin, or tobramycin) 1, 2
• Perform suitable challenge test when stable before starting inhaled antibiotics 1
• Consider cyclical IV antibiotics in patients with ≥5 exacerbations per year despite other treatments 1

For Non-Pseudomonas Frequent Exacerbators:

• Consider oral azithromycin 250-500mg three times weekly 1
• Rule out nontuberculous mycobacterial infection with at least one negative respiratory culture before starting long-term macrolides 1

Pseudomonas aeruginosa Eradication (First Isolation):

• First-line: Ciprofloxacin 500-750mg twice daily for 2 weeks 1
• Second-line: IV antipseudomonal beta-lactam ± IV aminoglycoside for 2 weeks, followed by 3 months of nebulized colistin, gentamicin, or tobramycin 1
• Send sputum for culture immediately before and at each clinical visit following eradication therapy 1

Bronchodilator Therapy

• Offer trial of short-acting bronchodilators before airway clearance and other inhaled therapies 1
• Consider long-acting bronchodilators (LABA, LAMA, or combination) in patients with significant breathlessness 1

Anti-Inflammatory Therapy

Do not routinely offer inhaled corticosteroids to patients with bronchiectasis without other indications (asthma, COPD, ABPA, inflammatory bowel disease). 1

• Do not offer long-term oral corticosteroids without specific indications 1
• Do not routinely offer PDE4 inhibitors, methylxanthines, leukotriene receptor antagonists, CXCR2 antagonists, neutrophil elastase inhibitors, or statins 1

Special Populations:

• Ensure optimal asthma control in patients with coexisting asthma; inhaled corticosteroids remain indicated 1, 3
• Consider trial of inhaled/oral corticosteroids in patients with inflammatory bowel disease and bronchiectasis 1
• For ABPA: consider itraconazole as steroid-sparing agent; monitor with total IgE levels 1

Pulmonary Rehabilitation

• Offer pulmonary rehabilitation (6-8 weeks of supervised exercise training) to all patients with impaired exercise capacity 1, 2
• Encourage regular physical exercise plus forced expiration technique/huff to promote airway clearance 1

Immunizations

• Offer annual influenza vaccination to all patients 1
• Offer 23-valent polysaccharide pneumococcal vaccination to all patients 1
• Consider 13-valent protein conjugate pneumococcal vaccine in patients without appropriate serological response to standard vaccine 1

Monitoring and Follow-Up

Assess patients annually at minimum, with more frequent monitoring (every 3-6 months) in severe disease. 1, 2

Patients Requiring Secondary Care Follow-Up:

• Chronic Pseudomonas aeruginosa, NTM, or MRSA colonization 1
• Recurrent exacerbations (≥3 per year) 1
• Declining lung function or deteriorating bronchiectasis 1
• Patients on long-term antibiotic therapy 1
• Associated conditions: rheumatoid arthritis, immune deficiency, inflammatory bowel disease, primary ciliary dyskinesia, ABPA 1

At Each Visit:

• Obtain sputum for culture and sensitivity 1, 2
• Perform pulse oximetry to screen for respiratory failure 1, 2
• Record weight and BMI 1
• Assess exacerbation frequency and symptom burden 2

Management of Complications

Hemoptysis:

• For ≤10mL over 24 hours: treat with appropriate oral antibiotic 1
• For major hemoptysis: multidisciplinary management with IV antibiotics based on known microbiology, consider tranexamic acid, and bronchial artery embolization as first-line intervention 1

Respiratory Failure:

• Consider long-term oxygen therapy using same eligibility criteria as COPD 1
• Consider domiciliary non-invasive ventilation with humidification for hypercapnic respiratory failure, especially with symptoms or recurrent hospitalization 1

Surgical Considerations

• Consider lung resection only in patients with localized disease whose symptoms are not controlled by optimized medical treatment 1, 2
• Requires multidisciplinary assessment including bronchiectasis physician, thoracic surgeon, and experienced anesthetist 1
• Consider transplant referral in patients ≤65 years if FEV1 <30% with significant clinical instability or rapid progressive deterioration despite optimal management 1
• Earlier transplant referral for: massive hemoptysis, severe secondary pulmonary hypertension, ICU admissions, or respiratory failure requiring NIV 1

Infection Control

• Individual or cohort segregation based on respiratory microbiology is not routinely required 1
• Apply good cross-infection prevention principles; if sharing facilities with CF patients, CF cross-infection guidelines should prevail 1

Critical Pitfalls to Avoid

• Never use antibiotic courses shorter than 14 days for exacerbations, especially with Pseudomonas aeruginosa 1
• Do not extrapolate CF bronchiectasis treatments to non-CF bronchiectasis, as treatment responses differ 1
• Do not discontinue inhaled corticosteroids in patients with coexisting asthma simply because bronchiectasis is present 1, 3
• Do not start long-term macrolides without ruling out NTM infection first 1
• Some patients may respond to antibiotics despite in vitro resistance; only change antibiotics if no clinical response 1