Bronchiectasis
Permanent structural dilation of airways due to chronic inflammation and recurrent infections.
- Massive hemoptysis (>200 mL/day).
- Hypoxemia (SpO2 < 88%) requiring supplemental O2.
Emergency Management: Acute respiratory failure or massive hemoptysis requiring immediate ICU admission and embolization.
Permanent, abnormal dilation and destruction of the major bronchi and bronchioles caused by chronic infection and inflammation.
Detailed Overview
Characterized by the vicious cycle of impaired mucociliary clearance, airway pooling of mucus, recurrent chronic bacterial infections, and intense neutrophilic inflammation. This destroys the elastin and muscular components of the bronchial wall. It typically presents with chronic daily cough with copious purulent sputum.
Epidemiology & Demographics
Prevalence is 350-500 per 100,000 in the US, rising steeply with age (>70 years). More common in women.
Etiological Mechanism
Post-infectious (severe pneumonia, TB), Cystic Fibrosis (CFTR mutations), Primary Ciliary Dyskinesia, and immunodeficiencies (CVID).
Primary Causes
Recurrent airway infections (Pseudomonas, H. influenzae, NTM) causing structural damage.
- Cystic Fibrosis: Most common cause of severe bronchiectasis in young patients.
- Immunodeficiency: Hypogammaglobulinemia prevents pathogen clearance.
An initial insult impairs mucociliary clearance. Retained mucus allows bacterial colonization (vicious cycle hypothesis). Neutrophils release elastase and matrix metalloproteinases, which destroy the muscularis and elastic tissue of the bronchial wall, leading to permanent abnormal dilation.
Characteristic Clinical Presentation
- Chronic Cough: Daily cough producing copious, foul-smelling, purulent sputum.
- Hemoptysis: Coughing up blood due to erosion into bronchial arteries.
Physical Examination Signs
- Crackles and Wheezes
- Digital Clubbing
- Massive Hemoptysis: Life-threatening bleeding from hypertrophied bronchial arteries.
- Cor Pulmonale: Right-sided heart failure due to chronic hypoxemia and pulmonary hypertension.
Diagnostic Criteria & Guidelines
Diagnosed radiographically by High-Resolution CT (HRCT) showing airway dilation (bronchoarterial ratio >1) and lack of airway tapering.
Differential Diagnosis
- COPD
- Asthma
- Chronic Rhinosinusitis
Laboratory Tests & Biomarkers
- Sputum Culture: Positive for H. influenzae, Pseudomonas aeruginosa, or NTM.
- Quantitative Immunoglobulins: Decreased IgG, IgA, IgM in CVID.
Imaging Modalities & Findings
- HRCT Chest:
-
Cylindrical
Uniform airway dilation.
-
Cystic
Balloon-like dilations ending in blind sacs.
Airway clearance therapy (chest physiotherapy, oscillatory PEP devices). Hypertonic saline (7%) nebulized BID. Treat acute exacerbations with oral Azithromycin 500 mg daily or Amoxicillin/Clavulanate 875/125 mg BID for 14 days.
Second-Line & Adjunctive Therapy
Inhaled antibiotics for chronic Pseudomonas colonization (e.g., Inhaled Tobramycin 300 mg BID alternating months). Chronic macrolide therapy (Azithromycin 250 mg 3x/week) for anti-inflammatory effects.
Surgical & Procedural Management
Bronchial artery embolization for massive hemoptysis. Surgical resection (lobectomy) for localized, severe disease failing medical therapy.
Recommended Lifestyle Changes
- Daily rigorous chest physiotherapy.
- Smoking cessation.
Patient Counseling & Advice
Emphasize that the structural lung damage is irreversible; daily mucus clearance is essential to prevent exacerbations.
Follow-Up & Monitoring Schedule
Annual spirometry (shows obstructive pattern, FEV1/FVC < 0.70) and semi-annual sputum cultures.
Preventive Strategies
Childhood vaccinations (pertussis, measles) and prompt treatment of pneumonia.
Progressive decline in lung function; FEV1 drops by 50-55 mL/year.
Frequently Asked Questions
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