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Non-neoplastic hamartomatous proliferation of atypical muscle cells that leads to progressive cystic destruction of lung parenchyma.

  • Five year survival of 50%
  • Exclusively women, mean age 34
  • Most do not have tuberous sclerosis, but when present it is usually obvious from childhood
  • Spontaneous pneumothorax
  • Round, thin wall cysts
  • Diffuse in distribution
  • Hyperinflated lungs
  • Not smoking related
  • Chylothorax

See Also

Eosinophilic granulomatosis