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Updated: Aug 21 2022


  • 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
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