Lymphangioleiomyomatosis

Lymphangioleiomyomatosis (LAM) is a rare lung disease that most commonly affects women in their mid-30s and 40s and causes the growth of cysts composed of smooth muscle and blood vessels in the lungs. These cysts can spread outside the lungs, forming noncancerous tumors on organs in the abdomen and pelvis. Often misdiagnosed as asthma or emphysema, most LAM sufferers experience shortness of breath, collapsed lung, chest pain, and fatigue. LAM is a progressive disease, and there is no known cure.

It is unclear what causes LAM, although estrogen seems to be a factor, because women rarely develop the disease before puberty or after menopause. Smoking isn’t known to cause LAM, as more than half of patients never smoked. Fewer than 2,000 women worldwide are known to have LAM today, including roughly 250 in the United States, and only about 10 men are known to have developed it. LAM is not cancer, but it shares some similarities with conditions that cause uncontrolled growth of benign tumors as well as another condition called tuberous sclerosis. A LAM diagnosis usually is confirmed through various types of imaging scans and pulmonary function tests.

UAB is home to one of only 24 LAM (lymphangioleiomyomatosis) clinics in the nation, as designated by the LAM Foundation. Offering a wide array of diagnostic and therapeutic services, the clinic provides multidisciplinary care for patients as well as support for their families. As part of The LAM Foundation Clinical Research Network, UAB conducts clinical trials to advance the discovery of improved treatments and prevention for this rare disease.

The LAM clinic is staffed by pulmonologists, radiologists, and pathologists who specialize in diagnosing LAM; interventional radiologists with expertise in smooth muscle tumor embolization; and cardiothoracic surgeons specializing in pleurodesis (preventing fluid buildup in the lungs) as well as lung transplants. Other essential members of the LAM clinic team include respiratory therapists, nutritionists, and physical therapists.

Resources

• Lung Transplant – Patient Resources
• Preparing for Your Lung Transplant
• Transplant Recovery at Hilton Birmingham at UAB
• Division of Pulmonary, Allergy and Critical Care Medicine