ER Medicine Quick Facts

The following medical information is provided as a general resource only. It is not intended to be used for patient education. It does not create any patient-physician relationship and should not be used as a substitute for professional diagnosis and treatment.

Lymphangioleiomyomatosis (LAM) is a rare lung disease in women that results in diffuse cystic changes in the lung parenchyma. It is associated with recurrent pneumothoraces, chylous pleural effusions, progressive respiratory failure and abdominal tumors, including lymphangiomyomas or angiomyolipomas, which can spontaneously hemorrhage and result in pain, anemia and hypotension.

Here are some insights for treating patients with LAM in the ER:

  • Pneumothorax should generally be managed with chest tube drainage followed by pleurodesis on the first event.
  • Cystic changes due to LAM are often not apparent on chest X ray. High-resolution CT scanning of the chest is the most definitive imaging test.
  • Pleural effusions usually represent chylothorax. This may be verified by thoracentesis and assessment of pleural fluid triglycerides and cholesterol.
  • Renal angiomyolipomas may bleed spontaneously producing flank pain, hematuria, hypotension and anemia.
  • Lymphangiomyomas may present as low-density abdominal lesions that mimic lymphoma or other malignancies.
  • If the patient is to undergo anesthesia, the anesthesiologist should be advised that the patient is at risk for a pneumothorax with positive pressure ventilation.
  • RAPAMUNE® (sirolimus) is an FDA approved drug to treat LAM. Special precautions should be taken for patients using sirolimus as it can impair wound healing in the event of urgent surgery. Sirolimus can also produce lung injury, immunosuppression, mouth ulcers, peripheral edema, acne like lesions and elevations of cholesterol.

The ICD 10 Code for LAM is J84.81.

For additional information or for a referral to a LAM specialist, contact The LAM Foundation at 513.777.6889.