Pulmonary Alveolar Proteinosis

Etiology

  • Interstitial and alveolar autoimmune (90%) lung disorder causing antibodies against GM-CSF
  • Low levels GM-CSF limits macrophage maturation, which then can't catabolize surfactant
  • Results in filling of alveoli w/lipid-rich surfactant and protein

Risk Factors

  • Rare
  • Median age 39
  • M > F
  • History of smoking
  • Inhalation exposure (silica, toxic fumes)

Symptoms

  • Sub-acute, progressive sob over months
  • Non-productive cough
  • No response to antibiotics (initial treatment often directed towards infection)

Physical Exam Findings

  • If advanced, abnormal vital signs, respiratory distress
  • Inspiratory crackles

Treatments

Tests