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  • Lymphangioleiomyomatosis

    Lymphangioleiomyomatosis (LAM) is a complex, rare lung disease that usually affects women of childbearing age. LAM triggers the body to make an abnormal amount of muscle cells which then deposit themselves in the organs of the body. Most commonly, these cells collect in the kidneys, lungs and lymph nodes. Because these cells do not perform the role of the organ they have collected in, the organ will not function efficiently and can cause serious complications for the patient. LAM in the lungs causes a decrease in the amount of oxygen that can be transferred out to the body, resulting in hypoxia, or decreased oxygen delivery to the organs.

    Causes

    There are two types of LAM, but both have an unknown cause. Most cases occur in women between the ages of 20 and 40, and there is a higher occurrence in women who have a condition known as tuberous sclerosis complex (TSC). About 30% of women with TSC will develop LAM, but it’s usually a milder form compared to women who develop the condition spontaneously.

    Symptoms

    Symptoms are triggered as the LAM cells begin to affect other organs of the body. Because a variety of other conditions may cause similar symptoms, testing will be needed to confirm the presence of LAM. The most common symptoms may include:

    • Wheezing
    • Chest pain or an aching chest
    • Shortness of breath that gets worse over time
    • Frequent cough with bloody mucus

    In more advanced cases:

    • Collapsed lungs (pneumothorax): lung cysts rupture through the lung creating an air leak
    • Pleural effusions: Collection of bodily fluids between the lung and chest wall, making it hard to take a full breath
    • Enlarged lymph nodes: Usually in the abdomen or chest
    • Bloody urine: May occur if the LAM cells have caused tumors in the kidneys
    • Swelling: in the legs, ankles, or feet

    Testing and diagnosis

    Providers will need to rule out other medical conditions such as asthma, bronchitis and emphysema to provide an LAM diagnosis. Patients may be treated by a pulmonologist depending on their case. Using a combination of patient history, diagnostic and lung function testing is the common approach. Tests that may be used include:

    • Lung function studies
    • Chest X-ray
    • Pulse oximetry
    • High resolution CT scan
    • Blood work
    • Lung biopsies
    • Thoracoscopy

    Treatment

    There is no treatment available to stop the growth of LAM cells in the body. Treatment focuses on managing symptoms and preventing complications.

    Some approaches used to treat LAM include:

    • Medications to open the lungs and move as much air as possible
    • Oxygen therapy
    • Procedures to remove fluid from the chest
    Lung transplantation
    • Procedures to shrink tumors in the kidneys, if present
    • Hormone therapy, if appropriate



Page last updated: 1/6/2014 4:08:26 PM