(See also Overview of Interstitial Lung Diseases Overview of Interstitial Lung Diseases Interstitial lung disease (also called diffuse parenchymal disease) is a term used to describe a number of different disorders that affect the interstitial space. The interstitial space consists... read more .)
Lymphangioleiomyomatosis (LAM) is rare. It occurs only in women, usually women between the ages of 20 and 40 years. The cause is unknown.
Affected women usually have shortness of breath. Sometimes cough, chest pain, and coughing up blood (hemoptysis) also occur. Symptoms may worsen during pregnancy. Sometimes the first indication of the disease is when a lung collapses (pneumothorax Pneumothorax A pneumothorax is the presence of air between the two layers of pleura (thin, transparent, two-layered membrane that covers the lungs and also lines the inside of the chest wall), resulting... read more 
) for no apparent reason. Sometimes fluid collects in the sac that covers the lungs (pleura).
The disorder tends to progress slowly, but eventually lung function deteriorates into respiratory failure Respiratory Failure Respiratory failure is a condition in which the level of oxygen in the blood becomes dangerously low or the level of carbon dioxide in the blood becomes dangerously high. Conditions that block... read more . The rate of progression varies widely, but progression may accelerate during pregnancy.
Diagnosis of Lymphangioleiomyomatosis
Chest computed tomography
Blood testing
A chest x-ray and computed tomography (CT) are usually needed for diagnosis of lymphangioleiomyomatosis.
A blood test that measures the level of vascular endothelial growth factor D (VEGF-D) is often done. VEGF-D levels are usually elevated in women who have LAM.
If the results of imaging and blood tests are unclear, doctors may remove pieces of lung tissue for examination under a microscope (lung biopsy).
Pulmonary function testing Pulmonary Function Testing (PFT) Pulmonary function tests measure the lungs' capacity to hold air, to move air in and out, and to absorb oxygen. Pulmonary function tests are better at detecting the general type and severity... read more 
shows that the amount of air the lungs can hold is below normal.
Treatment of Lymphangioleiomyomatosis
Sirolimus
Lung transplantation
Sirolimus, a drug normally used to suppress the immune system after a kidney transplant, seems to slow the decline in lung function in people with LAM.
Lung transplantation Lung and Heart-Lung Transplantation Lung transplantation is the surgical removal of a healthy lung or part of a lung from a living person and then its transfer into someone whose lungs no longer function. Heart-lung transplantation... read more may treat the disorder. However, sometimes LAM recurs in the transplanted lung.
More Information
The following is an English-language resource that may be useful. Please note that THE MANUAL is not responsible for the content of this resource.
LAM Foundation: General information on lymphangioleiomyomatosis, including research discoveries and support programs
Drugs Mentioned In This Article
| Generic Name | Select Brand Names |
|---|---|
sirolimus |
HYFTOR, Rapamune |
