What is Lymphangioleiomyomatosis?

LAM, or lymphangioleiomyomatosis (lim-FAN-je-o-LI-o-MI-o-ma-TO-sis), 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.

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 6 out of 10 women who have LAM develop pneumothorax (noo-mo-THOR-aks), or collapsed lung. In this condition, air leaks out of a lung and into the space between the lung and the chest wall (the pleural space).

This condition can cause pain and shortness of breath. Sometimes one lung will collapse over and over again. Pneumothorax is a serious condition that usually requires treatment and can sometimes be life threatening.

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

Image Source:NIH