What is bronchiectasis and how is it treated?

What is Bronchiectasis?

Bronchiectasis is a chronic respiratory disease characterized by permanent, irreversible dilation of the bronchi visible on CT scan, presenting clinically with chronic productive cough, recurrent respiratory infections, and sputum production. 1, 2

Pathophysiology

The disease operates through a self-perpetuating "vicious cycle" involving four interconnected components 1, 2:

• Chronic bacterial infection (most commonly Haemophilus influenzae, Pseudomonas aeruginosa, Moraxella catarrhalis, Staphylococcus aureus, and Enterobacteriaceae) 1, 2
• Neutrophilic inflammation that degrades airway elastin and causes rapid lung function decline 1, 2
• Impaired mucociliary clearance from structural damage, airway dehydration, and excessive mucus viscosity 1, 2
• Progressive structural lung damage with destruction of elastic and muscular components of bronchial walls 1, 3

Clinical Impact

The disease burden is substantial 1, 2:

• Quality of life impairment equals that of severe COPD and idiopathic pulmonary fibrosis 1, 2
• 50% of European patients experience ≥2 exacerbations annually 1, 2
• One-third require hospitalization each year 1, 2
• P. aeruginosa infection specifically confers 3-fold increased mortality risk, 7-fold increased hospitalization risk, and one additional exacerbation per year 1, 4

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Treatment of Bronchiectasis

Diagnostic Confirmation

High-resolution CT (HRCT) is the gold standard for confirming permanent bronchial dilatation and must be obtained before initiating treatment. 2, 3

Treatment Goals

The primary objectives are to prevent exacerbations, reduce symptoms, improve quality of life, and halt disease progression (lung function decline and mortality). 1, 2

Non-Pharmacological Management (First-Line)

Airway Clearance Techniques

All patients with chronic productive cough or difficulty expectorating should be taught airway clearance techniques by a trained respiratory physiotherapist. 2, 4

• Sessions should last 10-30 minutes, performed once or twice daily 4
• Techniques include active cycle of breathing, postural drainage, and manual or mechanical devices 2
• Consider intermittent positive pressure breathing or non-invasive ventilation during acute exacerbations 4

Pulmonary Rehabilitation

Patients with impaired exercise capacity should participate in pulmonary rehabilitation programs consisting of 6-8 weeks of supervised exercise training. 2, 4

• Benefits include improved exercise capacity, reduced cough symptoms, enhanced quality of life, and decreased exacerbation frequency 2, 4

Pharmacological Management

Bronchodilators

Offer a trial of long-acting bronchodilator therapy (LABA, LAMA, or combination) in patients with significant breathlessness, particularly those with chronic obstructive airflow limitation. 1, 4

• If treatment does not reduce symptoms, discontinue it 1
• Perform reversibility testing to identify co-existing asthma 4
• Follow COPD or asthma guideline recommendations for patients with these comorbidities 4

Mucoactive Treatments

Consider long-term mucoactive treatment for patients with difficulty expectorating sputum, poor quality of life, or failure of standard airway clearance techniques. 4

• Consider humidification with sterile water or normal saline to facilitate airway clearance 4
• Do NOT routinely use recombinant human DNase (dornase alfa) in adults with non-CF bronchiectasis 4, 1

Antibiotic Therapy for Acute Exacerbations

Treat exacerbations with 14 days of antibiotics, with selection based on previous sputum culture results. 4, 1

• Obtain sputum cultures before starting antibiotics whenever possible 4
• Common pathogens and recommended antibiotics 4:
  • Streptococcus pneumoniae: Amoxicillin 500mg TID (14 days)
  • Haemophilus influenzae (beta-lactamase negative): Amoxicillin 500mg TID (14 days)
  • Pseudomonas aeruginosa: Ciprofloxacin 500-750mg BID (14 days)
• Consider intravenous antibiotics for patients who are particularly unwell, have resistant organisms, or have failed to respond to oral therapy 4

Long-term Antibiotic Therapy

For patients with ≥3 exacerbations per year, consider long-term antibiotics. 2, 4

First-line treatments include:

• Long-term inhaled antibiotics (inhaled colistin or gentamicin) for patients with chronic Pseudomonas aeruginosa infection 2, 4
• Macrolides (azithromycin) for patients without Pseudomonas aeruginosa infection 2, 4

Anti-inflammatory Treatments

Do NOT routinely offer inhaled corticosteroids unless comorbid asthma or COPD is present. 4, 1

• Do not offer long-term oral corticosteroids without other indications, such as ABPA, chronic asthma, COPD, or inflammatory bowel disease 4
• For allergic bronchopulmonary aspergillosis (ABPA), immunosuppression with corticosteroids, with or without antifungal agents, is the mainstay of treatment 4

Treatment of Mycobacterium Avium Complex (MAC)

For severe or progressive MAC symptoms, treat with a macrolide (clarithromycin or azithromycin) with ethambutol and a rifamycin (rifabutin or rifampin) as first-line therapy. 1, 4

Immunizations

Offer annual influenza immunization to all patients with bronchiectasis. 4

Offer pneumococcal vaccination to all patients with bronchiectasis. 4

• Consider influenza vaccination in household contacts of patients with immune deficiency and bronchiectasis 4

Surgical Options

Surgery is NOT performed for adult patients with bronchiectasis except in cases of localized disease and high exacerbation frequency despite optimization of all other aspects of bronchiectasis management. 4

• Video-assisted thoracoscopic surgery (VATS) is often preferred to better preserve lung function and reduce scarring compared to open surgery 4
• Emergency surgery in unstable patients with massive hemoptysis is associated with higher morbidity and mortality reaching 37% 4

Lung Transplantation

Consider transplant referral in bronchiectasis patients aged 65 years or less if: 4

• FEV1 is <30% with significant clinical instability, OR
• Rapid progressive respiratory deterioration despite optimal medical management

Consider earlier transplant referral with additional factors such as: 4

• Massive hemoptysis
• Severe secondary pulmonary hypertension
• ICU admissions
• Respiratory failure

Key Clinical Pitfalls

• Underutilization of airway clearance techniques and pulmonary rehabilitation despite strong evidence 2, 4
• Failure to identify and treat P. aeruginosa infection aggressively given its dramatic impact on outcomes 2, 4
• Inadequate etiological workup missing treatable causes like immunodeficiency or ABPA 2, 3
• Extrapolating treatments from cystic fibrosis bronchiectasis, as treatment responses are different 4
• Using dornase alfa in non-CF bronchiectasis, which can worsen outcomes 4, 1