ILD

Langerhans Cell Granulomatosis

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Langerhans Cell Granulomatosis, Eosinophilic Granuloma, Pulmonary Histiocytosis X

  • Pathophysiology
  1. Dendritic Cell disorder
  • Related Conditions
  1. Letterer-Siwe
  2. Hand-Schuller-Christian Syndrome
  3. Histiocytosis X with Bone involvement
  • Epidemiology
  1. Ages 20-40 years old
  2. Prior Tobacco use (90%)
  • Complications
  1. Pneumothorax (10-30%)
  2. Diabetes Insipidus
  3. Lytic bone lesions (5-10%)
  • Management
  1. None available
  • Coarse
  1. Unpredictable prognosis
  2. Mortality 2-6%