Lung Disease & Respiratory Health Center

LAM Lung Disease (Lymphangioleiomyomatosis)

Diagnosis of LAM Lung Disease continued...

It's possible to diagnose LAM lung disease based on a woman's history and findings on high-resolution CT scan. But doctors often recommend collecting a sample of lung tissue (biopsy) to confirm the diagnosis. Lung biopsies can be collected in different ways:

• Bronchoscopy: An endoscope (flexible tube with a camera on its end) is passed into the windpipe and lower airways. Tools passed through the endoscope can collect a lung biopsy.
• Thoracoscopy: An endoscope is passed through a small incision into the chest to collect lung tissue.
• Open lung biopsy: Traditional surgery in which a surgeon works through a larger incision in the chest and takes a sample of lung tissue.

A physician (pathologist) then examines lung biopsy tissue, helping make the diagnosis of LAM lung disease with greater certainty.

Treatment of LAM Lung Disease

Unfortunately, no treatments have yet been shown to cure or improve pulmonary lymphangioleiomyomatosis.

Inhaled bronchodilators (albuterol, ipratropium) can help open the airways, reducing shortness of breath in some people.

Because hormones like estrogen seem to be involved in LAM lung disease, treatments that manipulate hormone levels could help some people with LAM. A number of hormone-related treatments have been tested in women with LAM lung disease:

• Progesterone
• Tamoxifen
• Synthetic luteinizing hormone-releasing hormones (Leuprolide, Lupron)

In clinical trials that tested these therapies, some women were helped, but others were not.

People who experience pneumothorax generally must undergo procedures to reinflate the collapsed lung, and prevent its recurrence.

If pulmonary lymphangioleiomyomatosis progresses and becomes disabling, lung transplantation may be an option. Though it's a drastic treatment, most people undergoing lung transplant for LAM lung disease gain improved lung function and quality of life after surgery.

What to Expect With LAM Lung Disease

Pulmonary lymphangioleiomyomatosis is progressive, and so far there is no cure. Most women with LAM experience a steady decline in lung function, with increased shortness of breath over time.

However, women vary widely in their experience of living with LAM lung disease. Some rapidly progress while others progress slowly: in some studies, nearly 90% of women were alive 10 years after diagnosis with LAM.

Researchers are working to identify how muscle cells go awry in LAM. Clinical trials of experimental drugs to treat LAM lung disease are also under way.