Lymphangioleiomyomatosis

  • Abnormal proliferation of interstitial smooth muscle cells, which obstruct small airways, causing air trapping and the formation of numerous thin-walled cysts.
  • The smooth muscle cell proliferation also causes lymphatic obstruction, which in turn may cause chylous fluid collections.
  • Exclusively affects women of child bearing age.
  • LAM is nearly identical to tuberous sclerosis-associated lung disease; however, intelligence is normal in patients with LAM.
  • Features of TS like angiomyolipoma of kidney and subependymal nodules can also be seen in LAM.
  • Radiographic features
    – In contrast to Langerhans cell histiocytosis, which has an upper lobe predominance, cysts in LAM are uniformly distributed, without sparing of costophrenic angle.
    – Cysts in LAM are also more regular in shape.
  • Other cystic lung diseases
    – TS-associated lung disease
    – LIP
    – LCH
    – PCP
  • Treatment: despite treatment with agents such as medroxyprogesterone and tamoxifen, LAM tends to be progressive with most of disease severity due to pulmonary disease.

Reference: radiopaedia.org

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