Lymphangioleiomyomatosis
Pronounced lim-fan je-o-lio-mi o-ma-to sis, Lymphangioleiomyomatosis (LAM) is a rare lung disease predominantly affecting women, most often occurring between puberty and menopause. In the United States, it is estimated that 1,000 women have LAM.
What is Lymphangioleiomyomatosis?
LAM is characterized by an unusual type of muscle cell that invades the tissue of the lungs. These muscle cells collect in the airway walls, causing them to become obstructed. Over time, the muscle cells block the flow of air, blood and lymph vessels to and from the lungs, preventing the lungs from providing oxygen to the rest of the body.
Causes
The cause of LAM is unknown.
Symptoms
At the onset of LAM, a patient may experience shortness of breath during strenuous exercise, but as the disease progresses, shortness of breath may even occur during rest. Other symptoms include chest pain, hemoptysis (coughing up blood), chylous effusions (leakage of white fluid into the chest cavity) and repeated pneumothoraces (leakage of air into the chest cavity).
Treatments
The diagnosis of LAM is difficult because early symptoms resemble those of lung disease. Because the cause is unknown, treatment to each diagnosis has been individual. Many treatments focus on reducing the production or effects of estrogen. Oxygen therapy may be necessary and a lung transplant may be considered as a last resort. Unfortunately, all treatments vary in effectiveness and none have been scientifically proven. A new drug, rapamycin, is currently being tested at the University of Cincinnati. To learn more about LAM and the current research in its diagnostics and treatment, visit the LAMfoundation website.
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What is Lymphangioleiomyomatosis?
LAM is characterized by an unusual type of muscle cell that invades the tissue of the lungs. These muscle cells collect in the airway walls, causing them to become obstructed. Over time, the muscle cells block the flow of air, blood and lymph vessels to and from the lungs, preventing the lungs from providing oxygen to the rest of the body.
Causes
The cause of LAM is unknown.
Symptoms
At the onset of LAM, a patient may experience shortness of breath during strenuous exercise, but as the disease progresses, shortness of breath may even occur during rest. Other symptoms include chest pain, hemoptysis (coughing up blood), chylous effusions (leakage of white fluid into the chest cavity) and repeated pneumothoraces (leakage of air into the chest cavity).
Treatments
The diagnosis of LAM is difficult because early symptoms resemble those of lung disease. Because the cause is unknown, treatment to each diagnosis has been individual. Many treatments focus on reducing the production or effects of estrogen. Oxygen therapy may be necessary and a lung transplant may be considered as a last resort. Unfortunately, all treatments vary in effectiveness and none have been scientifically proven. A new drug, rapamycin, is currently being tested at the University of Cincinnati. To learn more about LAM and the current research in its diagnostics and treatment, visit the LAMfoundation website.
Back to the Encyclopedia
