Bronchiectasis

Introduction

• A form of obstructive lung disease 
• Pathophysiology
  • Robust inflammatory response (neutrophil proteases)→ structural damage → mucous stasis → bacterial colonization → continued chronic inflammation
  • characterized bypermanent abnormal dilation of bronchi/bronchioles
  • ↓ of function of cartilage/elastin in conducting airways
• Causes
  • chronic infection
    • tuberculosis, H. influenzae, and S. aureus
    • atypical mycobacteria
  • bronchial obstruction
    • tumor, foreign body, and lymphadenopathy
  • cystic fibrosis
    • ↑ viscosity of mucus 
    • ↑ rate of respiratory infection due to mucus milleu
      • most common cause is P. aeruginosa
  • ↓ ciliary function 
    • primary ciliary dyskinesia (Kartagener syndrome)
      • genetic disease resulting in cilia without dyein arm
  • immunodeficiency
    • hypogammaglobulinemia and HIV
  • allergic bronchopulmonary aspergillosis (ABPA) 
  • autoimmune
    • RA and Sjogren’s syndrome
• α₁-antitrypsin deficiency

Presentation

• Symptoms
  • large amount of purulent sputum
  • recurrent infections
• hemoptysis

Evaluation

• CXR
  • “tram-track” lung markings due to bronchi extending to periphery
• CT 
•  thickened bronchial walls with dilated airways

Treatment

• Pharmacologic
  • airway clearance with nebulization (saline and hypertonic saline), postural drainage, percussion, and positive expiratory pressure device
  • azithromycin has been shown to decrease exacerbation rates
  • aggressive antibiotics for infections
    • attempts should be made to eradicate P. aeruginosa or MRSA
• Surgical
  • localized disease can be treated with lobectomy or segmentectomy
  • lung transplant
    • rare other than CF but could be considered if severe