Lymphangioleiomyomatosis, also known as LAM, is a type of interstitial lung disease that affects women almost exclusively.
The condition, characterized by the formation of cysts and abnormal growths on the lungs and other organs, typically strikes younger women between ages 20 to 40. While the average age of diagnosis is 35, older women in early menopause have also been diagnosed.
These growths make it difficult to breathe and put you at greater risk for other pulmonary issues, including respiratory failure.
That’s why it’s important to work with a team of interstitial lung disease specialists, like the ones at The Ohio State University Wexner Medical Center, to diagnose and treat LAM.
We’ve created an interstitial lung disease program to provide you with comprehensive and personalized care given by our pulmonary experts who have years of experience helping people with lung conditions like these.
What causes lymphangioleiomyomatosis?
There are two forms of LAM, and each has different causes:
- Sporadic LAM, which has no known cause
- Tuberous sclerosis LAM, which occurs in women with tuberous sclerosis, a rare genetic disease that causes tumors to form in various parts of the body
Symptoms of LAM
The abnormal growth of the smooth muscle cells and cysts on the lungs cause a blockage in the small airways, compromising the airflow and oxygen transfer to the blood. This can lead to respiratory issues.
Lymphangioleiomyomatosis is a progressive disease, meaning symptoms will worsen over time. In some people, this progression is slow and in others it’s much quicker.
LAM typically leads to decreased lung function, and many with the disease will require oxygen therapy at some point.
Other symptoms include:
- Shortness of breath
- Chest pains
- Coughing or wheezing
- Collapsed lung (pneumothorax)
- Pleural effusions (fluid in chest cavity)
- Blood in urine or when coughing
- Abdominal swelling
Diagnosing lymphangioleiomyomatosis
LAM is often misdiagnosed, since its symptoms mimic those of other respiratory illnesses, such as asthma or emphysema. Plus, it tends to manifest itself in different ways, depending on the person.
It’s important to work with a pulmonary expert at the Ohio State Wexner Medical Center to ensure you receive a proper diagnosis to dictate proper treatment.
We use a variety of tools to help us identify LAM as the cause of your symptoms and rule out other conditions. Those tests include:
- Medical exam and history
- Lung function tests – Devices, like a spirometer, allow us to measure how well your lungs work.
- High-resolution CT scan – This advanced imaging test will allow us to see if there are cysts on the lungs, which is the main sign of LAM. We can also see if there is fluid around the lungs.
- Blood tests – These look at a variety of disease markers, including elevated levels of a hormone called VEGF-D, which can confirm LAM without a biopsy.
- Lung biopsy – Sometimes examining a small piece of lung tissue is required to make a definitive diagnosis.
- MRI and other imaging tools – We see if you have tumors in the kidneys or cysts in other parts of the body, which are also common in LAM.
Lymphangioleiomyomatosis treatment
Recent advancements in medicine have allowed those with the disease to live longer and have a better quality of life.
While there is no cure, we can often prevent LAM from progressing and stabilize lung function, allowing you to continue many of your favorite daily activities.
We mainly do this using a drug called sirolimus (rapamycin), which not only can improve lung function but also shrink kidney tumors related to LAM.
Other types of treatments include:
- Medications – Besides sirolimus, there are other drugs we can prescribe, such as bronchodilators and medicine to prevent bone loss.
- Oxygen therapy – This can help make breathing easier.
- Hormone therapy – Estrogen is believed to contribute to the growth of LAM cells.
- Procedures – There are certain procedures we can do to drain fluid from the lungs and shrink tumors, both of which could ease symptoms.
- Lung transplant – If your case is severe, we might recommend a lung transplant. This won’t cure the disease, but it can add years to your life.