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Updated: Apr 28 2021


  • Introduction
    • A condition characterized bydilation of air spaces (with ↓ elasticity and ↑ compliance)
      • due to alveolar wall destruction
        • normally, elastin in the wall functions to keep alveoli open
      • capillaries are destroyed along with the alveoli
    • Causes
      • smoking
        • most common cause
      • α1-antitrypsin (AAT) deficiency
        • AD disorder (codominant)
          • MM phenotype is normal
          • ZZ phenotype results in disease
        • AAT normally inhibits the action of elastase
        • without AAT elastase is unchecked and destroys the elastic tissue of the alveoli
        • as a result emphysema develops at early age
          • may also develop cirrhosis due to the inability to release an abnormal form of AAT from the liver resulting in hepatotoxicity
  • Classification
    • Centriacinar
      • dilated respiratory bronchiole
      • most common presentation of emphysema due to smoking
        • result of inhaled tobacco toxins arriving first in the respiratory bronchioles before traveling to the alveolus
      • most commonly in the upper lobes
        • result of upper lobes receiving exposure to smoke
    • Panacinar
      • dilated alveoli
      • most common presentation of AAT deficiency
        • also due to a functional AAT deficiency as a result of smoking
          • oxidants and inflammatory reaction of smoke can destroy AAT
          • smoking exacerbates effects of genetic AAT deficiency
      • most commonly in the lower lobes
        • result of lower lobes recieving ↑ perfusion allowing more immune cells to traffic into the alveoli
    • Paraseptal
      • most commonly involves young, otherwise healthy males
      • does not obstruct the airway
        • associated with bullae
        • found near the pleura
        • increased risk for spontaneous pneumothorax
  • Presentation
    • Symptoms
      • dyspnea
      • classic pursed-lip breathing
        • results in increased airway pressure and prevents airway collapse during exhalation
    • Physical exam
      • decreased breath sounds on auscultation
      • increased anterior-posterior diameter (barrel chest)
      • hyperresonant to percussion
      • "pink puffer" (end-stage)
  • Imaging
    • Chest radiograph
      • increased AP diameter with flattened diaphragms
      • hyperinflated lungs
      • loss of lung markings
      • elongated heart
  • Evaluation
    • Labs
      • ABG during exacerbation shows hypoxemia and acute respiratory acidosis
      • AAT shows no α-globin peak on electrophoresis
    • Pulmonary function tests
      • decreased FEV1 sec / FVC
      • decreased diffusion capacity from destruction of capillaries
  • Treatment
    • Conservative
      • smoking cessation
      • ambulatory O2
    • Pharmacologic
      • bronchodilators
        • for symptom improvement
      • inhaled steroids
        • reduce exacerbations
      • oral/IV steroids and antibiotics
        • for acute exacerbations
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