Bronchiectasis
Critical Alerts
- Massive hemoptysis can occur and requires emergent management
- Pseudomonas aeruginosa is common - consider antipseudomonal coverage
- Respiratory failure may develop in severe exacerbations
- Chronic colonization differs from acute infection - know patient's baseline
- Non-tuberculous mycobacteria (NTM) may be present - consider if not responding
Key Diagnostics
- Chest X-ray (may show bronchial wall thickening, "tram tracks")
- CT chest (gold standard for diagnosis - bronchial dilation)
- Sputum culture including AFB (guide antibiotic therapy)
- CBC, CRP (inflammatory markers)
- ABG/VBG if respiratory distress
Emergency Treatments
- Oxygen therapy: Target SpO2 88-92% (may have chronic hypercapnia)
- Antibiotics: Empiric based on prior cultures; cover Pseudomonas if colonized
- Bronchodilators: Nebulized salbutamol and ipratropium
- Airway clearance: Physiotherapy, nebulized hypertonic saline
- Hemoptysis management: Tranexamic acid, bronchial artery embolization if massive
Bronchiectasis is a chronic respiratory disease characterized by irreversible, abnormal dilation of the bronchi with impaired mucociliary clearance, chronic infection, and inflammation. Patients experience recurrent respiratory infections and progressive lung damage.
Pathological Classification
| Type | Description |
|---|---|
| Cylindrical | Uniform dilation of bronchi (most common) |
| Varicose | Irregular dilation with beaded appearance |
| Cystic/Saccular | Severe dilation with sac-like structures (most severe) |
Epidemiology
- Prevalence: 50-500 per 100,000 (increasing with CT availability)
- Age: Can occur at any age; prevalence increases with age
- Sex: Female predominance in non-CF bronchiectasis
- Mortality: Increased compared to general population
Etiology
| Category | Causes |
|---|---|
| Post-infectious | Childhood pneumonia, TB, pertussis, measles |
| Immunodeficiency | Common variable immunodeficiency, HIV, IgG subclass deficiency |
| Genetic | Cystic fibrosis, primary ciliary dyskinesia, alpha-1 antitrypsin |
| Aspiration | GERD, neurological conditions |
| Autoimmune | Rheumatoid arthritis, Sjögren's syndrome |
| Allergic | ABPA (allergic bronchopulmonary aspergillosis) |
| Obstruction | Foreign body, tumor |
| Idiopathic | Up to 50% of cases |
Vicious Cycle Hypothesis
Cole's Vicious Cycle
Initial insult (infection, obstruction, immune defect)
↓
Impaired mucociliary clearance
↓
Bacterial colonization
↓
Chronic inflammation (neutrophils, cytokines)
↓
Airway damage and dilation
↓
Further impairment of clearance
↓
More infection and inflammation (cycle repeats)
Microbiology
Common Colonizing Organisms
| Organism | Features |
|---|---|
| Haemophilus influenzae | Most common; non-typeable strains |
| Pseudomonas aeruginosa | Associated with worse outcomes; difficult to eradicate |
| Streptococcus pneumoniae | Common in exacerbations |
| Moraxella catarrhalis | Common, usually less virulent |
| Staphylococcus aureus | Consider in CF or post-viral |
| Non-tuberculous mycobacteria | MAC, M. abscessus - difficult to treat |
| Aspergillus | ABPA, colonization |
Complications
- Recurrent exacerbations
- Progressive lung function decline
- Respiratory failure
- Massive hemoptysis
- Cor pulmonale
- Amyloidosis (rare)
Chronic Symptoms
| Symptom | Frequency | Description |
|---|---|---|
| Chronic productive cough | 90%+ | Daily sputum production, often purulent |
| Dyspnea | 70-80% | Progressive, worse with exacerbations |
| Fatigue | Common | Related to chronic infection |
| Hemoptysis | 30-50% | Usually mild; can be massive |
| Chest pain | 20-30% | Pleuritic or related to cough |
| Recurrent infections | Hallmark | Multiple courses of antibiotics/year |
Exacerbation Definition
Acute Exacerbation of Bronchiectasis Deterioration in ≥3 of the following for ≥48 hours:
Physical Examination
General
Respiratory
| Finding | Significance |
|---|---|
| Coarse crackles | Secretions in dilated airways |
| Wheeze | Airflow obstruction |
| Reduced breath sounds | Severe disease, consolidation |
| Increased AP diameter | Air trapping |
Severity Assessment
BSI (Bronchiectasis Severity Index)
Critical Presentations
| Red Flag | Concern | Action |
|---|---|---|
| Massive hemoptysis (>00mL/24h) | Bronchial artery rupture | Emergent bronchial artery embolization |
| Respiratory failure (SpO2 <88%) | Severe exacerbation | ICU, NIV/intubation consideration |
| Sepsis | Severe infection | Sepsis protocol, broad antibiotics |
| New oxygen requirement | Significant decline | Admission, workup |
| Confusion/altered consciousness | Hypercapnia, sepsis | ABG, ICU consideration |
| Persistent fever despite antibiotics | Resistant organism, abscess | CT, consider NTM/fungi |
Massive Hemoptysis Management
Immediate Actions:
1. Position bleeding lung down (if known)
2. Protect airway - suction
3. Large-bore IV access
4. Type and crossmatch
5. Tranexamic acid 1g IV
6. Urgent CXR (lateralize source)
7. Call for help - ICU, interventional radiology, thoracic surgery
Definitive Treatment:
- Bronchial artery embolization (first-line)
- Bronchoscopy for localization
- Surgery if embolization fails
Conditions with Similar Presentations
| Condition | Distinguishing Features |
|---|---|
| COPD | Smoking history, emphysema on CT, no bronchial dilation |
| Asthma | Reversible obstruction, no structural changes |
| Chronic bronchitis | Smoking related, no bronchiectasis on CT |
| Tuberculosis | AFB positive, cavitary lesions |
| Lung cancer | Mass on imaging, hemoptysis, weight loss |
| Interstitial lung disease | Restrictive pattern, fibrosis on CT |
| Cystic fibrosis | Younger onset, pancreatic insufficiency, sweat test |
Causes of Chronic Productive Cough
- Bronchiectasis
- COPD
- Chronic sinusitis with post-nasal drip
- GERD-related cough
- ACE inhibitor-related cough
- Lung cancer
Clinical Assessment
Key History
- Duration and characteristics of symptoms
- Sputum volume and color daily vs during exacerbation
- Prior cultures and antibiotic sensitivities
- Exacerbation frequency
- Prior hospitalizations
- Known colonizing organisms
- Baseline pulse oximetry and FEV1
Laboratory Studies
| Test | Purpose | Findings |
|---|---|---|
| Sputum culture | Guide antibiotic therapy | May grow colonizing organisms |
| AFB smear/culture | NTM, TB | Rule out mycobacterial disease |
| CBC | Infection, anemia | Leukocytosis, anemia of chronic disease |
| CRP | Inflammatory marker | Elevated in exacerbation |
| ABG/VBG | Respiratory failure | Hypoxemia, hypercapnia |
| Procalcitonin | Bacterial infection | May help guide antibiotics |
Imaging
Chest X-ray
- May be normal in mild disease
- "Tram track" sign (thickened bronchial walls)
- Ring shadows (dilated bronchi en face)
- Air-fluid levels in dilated bronchi
- Consolidation if infection
High-Resolution CT (HRCT) - Gold Standard
| Finding | Description |
|---|---|
| Bronchial dilation | Internal diameter > adjacent artery (signet ring) |
| Lack of tapering | Bronchi visible in outer third of lung |
| Bronchial wall thickening | >mm |
| Tree-in-bud opacities | Small airway disease |
| Mucus plugging | Air-fluid levels in airways |
Pulmonary Function Tests
- Often obstructive pattern
- FEV1/FVC <0.7
- Reduced FVC in advanced disease
- Air trapping (increased RV/TLC)
Acute Exacerbation Management
Antibiotic Selection
| Colonization Status | Empiric Regimen | Duration |
|---|---|---|
| No prior cultures | Amoxicillin-clavulanate 875/125mg PO BID | 14 days |
| H. influenzae | Amoxicillin-clavulanate or doxycycline | 14 days |
| Pseudomonas | Ciprofloxacin 750mg PO BID or IV antipseudomonal | 14 days |
| MRSA | TMP-SMX or doxycycline | 14 days |
| Severe/hospitalized | IV piperacillin-tazobactam or ceftazidime + aminoglycoside | 14 days |
Key Principle: Use prior culture data to guide therapy
Bronchodilators
- Nebulized salbutamol 2.5-5mg q4-6h
- Nebulized ipratropium 500mcg q6h
- Consider if wheeze or airflow obstruction
Airway Clearance
- Chest physiotherapy
- Active cycle of breathing technique
- Nebulized hypertonic saline (3-7%) - loosens secretions
- Mucolytics (dornase alfa only in CF; others unproven)
Oxygen Therapy
- Target SpO2 88-92% (may have chronic hypercapnia)
- Avoid high-flow oxygen if CO2 retainer
- Check ABG if concern for hypercapnia
Hemoptysis Management
Minor Hemoptysis (<100mL/24h)
- Reassurance
- Treat underlying infection
- Consider tranexamic acid 500mg PO TID
- Avoid chest physiotherapy during active bleeding
Moderate/Massive Hemoptysis
- As per red flag management above
- Tranexamic acid 1g IV
- Bronchial artery embolization
- Rigid bronchoscopy for airway control if needed
Non-Invasive Ventilation
Indications
- Hypercapnic respiratory failure
- pH <7.35 with PaCO2 >45 mmHg
- Severe respiratory distress
Settings
- IPAP 12-20 cmH2O
- EPAP 4-6 cmH2O
- Titrate to patient comfort and ABG response
Chronic Management (Outpatient)
| Intervention | Purpose |
|---|---|
| Airway clearance (daily) | Reduce sputum stasis |
| Inhaled bronchodilators | Improve airflow, aid clearance |
| Pulmonary rehabilitation | Improve exercise tolerance |
| Macrolide prophylaxis | Reduce exacerbations (azithromycin 250mg 3x/week) |
| Vaccinations | Influenza, pneumococcal, COVID-19 |
| Nebulized tobramycin | Pseudomonas eradication/suppression |
Admission Criteria
- Hypoxia requiring supplemental oxygen
- Severe exacerbation with respiratory distress
- Failure of outpatient antibiotics
- Inability to manage at home
- Significant hemoptysis
- Need for IV antibiotics
- Comorbidities complicating management
ICU Admission Criteria
- Respiratory failure requiring NIV or intubation
- Massive hemoptysis
- Sepsis or hemodynamic instability
- Hypercapnia with pH <7.25
Discharge Criteria
- Stable or improving symptoms
- Oxygen at or near baseline
- Able to take oral medications
- Airway clearance education provided
- Follow-up arranged
- Exacerbation action plan in place
Follow-up Recommendations
| Timeframe | Purpose |
|---|---|
| 2-4 weeks | Post-exacerbation pulmonology review |
| Sputum culture | During exacerbation for future guidance |
| Annual | Pulmonary function tests, CT if indicated |
Understanding Bronchiectasis
- Bronchiectasis is a chronic lung condition with widened airways
- Airways become damaged and prone to repeated infections
- Daily airway clearance is essential
- Complete antibiotic courses as prescribed
Daily Management
Airway Clearance
- Perform twice daily (more during exacerbations)
- Techniques include huff coughing, active cycle breathing
- Use hypertonic saline nebulizer if prescribed
- Devices like flutter valves or vest therapy may help
Hydration
- Good hydration helps thin secretions
- Aim for 2-3L fluid daily unless restricted
Exacerbation Recognition
Seek care if:
- Increased sputum volume or color change
- Increased breathlessness
- New or increased fever
- Worsening fatigue
- Blood in sputum
Prevention
- Annual influenza vaccination
- Pneumococcal vaccination
- COVID-19 vaccination
- Avoid respiratory irritants
- Good hand hygiene
- Treat GERD if present
Cystic Fibrosis-Related Bronchiectasis
- Earlier onset, more severe
- Pseudomonas colonization very common
- Pancreatic insufficiency, diabetes, sinusitis
- CF transmembrane conductance regulator (CFTR) modulators
- Dornase alfa (Pulmozyme) effective for sputum
Immunodeficiency-Associated
- Screen for immunoglobulin deficiency
- IgG replacement therapy if deficient
- More aggressive infection management
Non-Tuberculous Mycobacterial Infection
Suspect if:
- Not responding to standard antibiotics
- Nodular or cavitary disease
- Low BMI, older female
- MAC is most common
Management:
- Multiple drug regimen (macrolide + rifampin + ethambutol)
- Prolonged treatment (12+ months after culture negative)
- Specialist involvement essential
ABPA (Allergic Bronchopulmonary Aspergillosis)
- Sensitization to Aspergillus in airway
- Eosinophilia, elevated IgE
- Central bronchiectasis characteristic
- Treatment: Oral corticosteroids, itraconazole
Performance Indicators
| Metric | Target |
|---|---|
| Sputum culture sent during exacerbation | >0% |
| Antibiotic selection based on prior cultures | >0% |
| Oxygen saturation documented | 100% |
| Airway clearance education provided | 100% |
| Follow-up arranged | 100% |
| Vaccination status reviewed | 100% |
Documentation Requirements
- Baseline pulmonary status (FEV1, usual SpO2)
- Prior cultures and sensitivities
- Current symptoms vs baseline
- Antibiotic choice rationale
- Airway clearance plan
- Discharge instructions
Diagnostic Pearls
- CT chest is gold standard - don't rely on CXR alone
- Know the patient's baseline - colonization vs acute infection
- Always send sputum during exacerbations
- Consider NTM if not responding to standard therapy
- Screen for underlying causes - immunodeficiency, CF, ABPA
Treatment Pearls
- Pseudomonas requires specific coverage - ciprofloxacin or IV antipseudomonal
- 14 days of antibiotics is standard for exacerbations
- Airway clearance is as important as antibiotics
- Avoid over-oxygenation in chronic CO2 retainers
- Tranexamic acid is first-line for hemoptysis
Disposition Pearls
- Low threshold for admission in severe exacerbation
- Ensure baseline is documented for future reference
- Follow-up is essential - chronic disease requires continuity
- Exacerbation action plan empowers patients
- Pulmonology referral for new diagnosis or frequent exacerbations
- Polverino E, et al. European Respiratory Society guidelines for the management of adult bronchiectasis. Eur Respir J. 2017;50(3):1700629.
- Hill AT, et al. British Thoracic Society Guideline for bronchiectasis in adults. Thorax. 2019;74(Suppl 1):1-69.
- Martinez-Garcia MA, et al. Multidimensional approach to non-cystic fibrosis bronchiectasis: the FACED score. Eur Respir J. 2014;43(5):1357-1367.
- Chalmers JD, et al. The bronchiectasis severity index. Am J Respir Crit Care Med. 2014;189(5):576-585.
- King PT. The pathophysiology of bronchiectasis. Int J Chron Obstruct Pulmon Dis. 2009;4:411-419.
- Pasteur MC, et al. British Thoracic Society guideline for non-CF bronchiectasis. Thorax. 2010;65 Suppl 1:i1-58.
| Version | Date | Changes |
|---|---|---|
| 1.0 | 2025-01-15 | Initial comprehensive version with 14-section template |