Key Points

• LAM, or lymphangioleiomyomatosis, is a rare lung disease. It mostly affects women in their mid-thirties and forties.
• In LAM, abnormal, muscle-like cells begin to grow out of control in certain organs or tissues, especially the lungs, lymph nodes, and kidneys.
• Over time, these LAM cells can grow throughout the lungs and destroy the normal lung tissue. As a result, air can't move freely in and out of the lungs. In some cases, this means the lungs can't supply the body's other organs with enough oxygen.
• LAM also can lead to pneumothorax (collapsed lung), angiomyolipomas (AMLs), growths in certain organs, large tumors in the lymph nodes, and a buildup of fluid in the chest, abdomen, or pelvic area.
• There are two forms of LAM. Sporadic LAM occurs for unknown reasons. LAM also can occur in women who have a rare disease called tuberous sclerosis complex (TSC). Women who have TSC often have a milder form of LAM.
• The cause of LAM and why it mainly affects women isn't known. Sporadic LAM has some of the same traits as TSC. The abnormal genes that cause TSC also play a role in causing LAM.
• Except for rare cases, LAM only affects women. More than 70 percent of women who develop LAM are between the ages of 20 and 40 when they begin to have symptoms. LAM affects about 3 out of every 10 women who have TSC.
• The uncontrolled growth of LAM cells and their effect on nearby body tissues causes the signs and symptoms of LAM. The most common signs and symptoms are shortness of breath, especially during activity; chest pain or aches; frequent cough; and wheezing.
• Methods for diagnosing LAM have improved. It's now possible to diagnose the disease at an early stage. LAM is diagnosed based on your signs and symptoms and the results from tests and procedures.
• Currently, no treatment is available to stop the growth of the cysts and cell clusters that LAM causes. Most treatments for LAM are aimed at easing symptoms and preventing complications. Treatments include medicines, oxygen therapy, procedures to remove fluid from the chest or abdomen, procedures to remove AMLs, lung transplant, and hormone therapy.
• An experimental medicine, rapamycin (sirolimus), shows promise in shrinking tumors in the kidneys of women who have LAM. Studies are under way to find out whether this medicine improves lung function in women who have LAM.
• In the early stages of LAM, you usually can do your normal daily activities. In the later stages of LAM, it may be harder for you to be active. You also may need oxygen therapy full time. If you have LAM, you can take steps to care for yourself. Get ongoing medical care. Following a healthy lifestyle, including a healthy eating plan.
• Researchers continue to look for the causes of LAM and for better treatments.