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Lymphangioleiomyomatosis (LAM)

Lymphangioleiomyomatosis or LAM, is a rare cystic lung disease that mostly affects women in their mid-forties. In LAM, an unusual type of cell begins to grow out of control throughout the body, including in the lungs, lymph nodes and vessels, and kidneys

Prevalence

1-9/100,000

331-2,979

US Estimated

513-4,622

Europe Estimated

Age of Onset

Adult

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ICD-10

D48.7

Inheritance

This condition does not appear to have a clear pattern of inheritance.

5 Facts you should know

FACT

1

A rare lung disease that mainly affects women, usually during their childbearing years

FACT

2

Lymphangioleiomyomatosis (LAM) occurs in 3-8 women per million in the general population 

FACT

3

There are 2 forms of LAM - a sporadic form, which occurs for unknown reasons, and a form that occurs in people with tuberous sclerosis complex

FACT

4

It typically results in cystic lung destruction

FACT

5

Diagnosis is typically delayed 5 to 6 years because it’s often diagnosed as asthma or chronic obstructive pulmonary disease (COPD)

Lymphangioleiomyomatosis is also known as...

Lymphangioleiomyomatosis is also known as...

  • LAM​ disease
  • Lymphangio-myomatosis

What’s your Rare IQ?

Which medication is commonly used to treat LAM?

Common signs and symptoms

Chest pain

Cough

Dyspnea

Pulmonary infiltrates

Current treatments

While there is currently no cure for LAM, research has led to major progress in the treatment of symptoms. Treatment options may vary from person to person depending on the severity of the disease. People with LAM should speak with their doctor about whether certain options are right for them, as well as the benefits and risks of treatment options.

Sirolimus (also called rapamycin)

Oxygen therapy

Bronchodilators

Lung transplant