Updated: 10/9/2019

Bronchiectasis

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Introduction
  • A form of obstructive lung disease 
  • Pathophysiology
    • Robust inflammatory response (neutrophil proteases)→ structural damage → mucous stasis → bacterial colonization  continued chronic inflammation
    • characterized by permanent 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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