LAM or Lymphangioleiomyomatosis

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LAM, or lymphangioleiomyomatosis (lim-FAN-je-o-LI-o-MI-o-ma-TO-sis), is a rare lung disease that mostly affects women of childbearing age.

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 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.

Overview

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.

About 50 percent of women who have
LAM develop pneumothorax (noo-mo-THOR-aks), or collapsed lung. In this condition, air leaks out of the lung and into the space between the lung and chest wall (the pleural space).

A collapsed lung can cause pain and shortness of breath. Sometimes one lung will collapse over and over again. Pneumothorax is a serious condition. It usually requires treatment and might be life threatening.

Normal Lungs and Lungs With LAM

Figure A shows the location of the lungs and airways in the body. The inset image shows a cross-section of a healthy lung. Figure B shows a view of the lungs with LAM and a collapsed lung (pneumothorax). The inset image shows a cross-section of a lung with LAM.

Many women who have LAM get tumors called angiomyolipomas (AN-je-o-my-o-li-PO-mas), or AMLs, in their kidneys. Women who have LAM also may develop:

• Growths in other organs, including the liver and brain
• Large tumors in their lymph nodes
• A buildup of fluid in their chests, abdomens, or pelvic areas

Outlook

LAM has no cure, and the disease tends to worsen over time. How quickly the disease worsens varies from woman to woman. LAM may lead to death from respiratory failure. Lung transplant is a treatment option for women whose lungs have been damaged by LAM.

Not long ago, doctors thought women who had LAM wouldn't live more than 8–10 years following diagnosis. They now know that some women may survive longer (as long as 20 years following diagnosis, although this is rare).

Doctors have learned a lot about LAM in recent years. They're now able to diagnose the condition earlier. Support services also are now available to help improve the quality of life for women who have LAM.

Researchers continue to explore and test new treatments for LAM.