• A rare lung disease that tends to affect women of childbearing age.
  • The muscle cells that line the lung’s airways and blood vessels begin to multiply abnormally. These muscle cells spread into areas of the lung where they don’t belong.
  • The air sacs in the lung also swell and form small pockets called cysts. As the cysts develop throughout the lungs, LAM causes breathing problems similar to emphysema.
  • The muscle cells can spread outside the lung, forming noncancerous tumors on organs in the abdomen and pelvis.
  • Cause is unknown
    • Estrogen seems to be involved, because women rarely develop LAM before puberty or after menopause. Fewer than 10 men are known to have ever developed LAM lung disease.
    • Smoking isn’t known to cause LAM: more than half of people with LAM lung disease have never smoked.
  • LAM is so rare that it’s unknown how often it really occurs.
  • LAM is not cancer, but appears similar to other conditions in which benign tumors grow uncontrollably. LAM lung disease shares some features with a different condition called tuberous sclerosis.
  • Most people with LAM lung disease experience shortness of breath. Other symptoms include wheezing and cough, which may be bloody.
  • Often, people with LAM develop a sudden pneumothorax (collapsed lung). This occurs when one of the cysts near the lung’s edge ruptures, allowing inhaled air to compress the lung. A pneumothorax usually causes pain and shortness of breath.
  • If muscle cells migrate to areas outside the lungs, LAM can cause other symptoms:
    • Chylous ascites: The flow of lymph is blocked by errant muscle cells. Chyle (milky lymph fluid) builds up in the belly.
    • Angiomyolipomas: Noncancerous tumors may grow in the liver or kidneys. These can cause pain, bleeding, or kidney failure.
      • In a few people with LAM, discovery of these benign tumors outside the lungs is the first sign of LAM lung disease.


  • Physician may do/request:
    • History & Physical Exam
  • Other additional tests such as:
    • Chest X-ray:
    • Pulmonary function tests
    • Computed tomography (CT scan)
    • Lung Biopsy collected through:
      • Bronchoscopy
      • Thoracoscopy
      • Open lung biopsy



  • Sirolimus (Rapamune)
    • The first drug approved to treat lymphangioleiomyomatosis.
      • Helps improve the lung capacity in patients, allowing them to breathe easier.
    • Inhaled bronchodilators(albuterol, ipratropium)
    • Hormone-related treatments may help, such as:
      • Progesterone
      • Tamoxifen
      • Synthetic luteinizing hormone-releasing hormones (Leuprolide, Lupron)
    • Lung transplant
      • For severe cases

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