Pathology

Lymphangioleiomyomatosis (LAM): A very rare lung disease



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Lymphangioleiomyomatosis, commonly known as LAM, is a very rare and serious lung disease. Lymphangioleiomyomatosis begins with shortness of breath during exercise and progresses to the point where breathing becomes difficult throughout the day. LAM mostly affects women, though men can also develop the condition. Diagnosing LAM is a work in progress due to the rarity of the condition and the fact that its symptoms are similar to other lung conditions.

According to the Mayo Clinic, lymphangioleiomyomatosis behaves like a cancer, causing abnormal cells to multiply and develop cysts that interfere with normal tissue function. Doctors experience difficulty with diagnosing LAM because its symptoms often mimic more common respiratory diseases like asthma, bronchitis, and chronic obstructive pulmonary disease (COPD).

Symptoms of LAM can assist in its diagnosis. In addition to difficulty breathing, LAM causes chest pain and coughing up blood. LAM is more likely to be suspected in women. However, while women are far more likely to develop LAM, this is not to say that they face a significant risk. In fact, the University of Louisville estimates that the number of women in the U. S. who have LAM likely sits in the hundreds.

In the early stages of the condition, LAM is often misdiagnosed. WebMD reports that X-rays often show no abnormalities until the disease progresses. Computed tomography (CT) scans are more effective at shedding light on damage caused by LAM. Blood testing can be used to examine how much oxygen is reaching the blood. According to the University of Louisville, once a doctor suspects LAM, a lung biopsy proves most effective at confirming the diagnosis.

With no known cure, treatments for lymphangioleiomyomatosis aim to manage the symptoms of the condition. According to the National Institutes of Health, bronchodilators which treat asthma also improve breathing for about one-quarter of women with LAM. Depending on the severity of the condition, particularly regarding blood-oxygen levels, oxygen therapy is often used to manage LAM. Oxygen therapy is commonly used during physical activity, though eventually the body requires it more extensively.

Estrogen is believed to play a role in LAM progression and some treatments aim to neutralize its effects. WebMD reports that progesterone and tamoxifen sometimes demonstrate effectiveness, though results vary from patient to patient. According to the National Institutes of Health, rapamycin sometimes improves lung function, though again the results vary and it comes with a number of side effects. In the worst cases of the condition, LAM sometimes causes a lung to collapse. This can require surgery or even lung transplant.

Lymphangioleiomyomatosis is a lung disease that causes difficulty breathing, coughing and chest pain. LAM is very rare, afflicting only a few hundred Americans. As a result, it is prone to misdiagnosis. Treatments for LAM center around limiting symptoms and curbing the spread of abnormal tissue growth. Talk to your doctor if you have any questions about LAM, its diagnostic procedures, or its treatments.

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ARTICLE SOURCES AND CITATIONS
  • InfoBoxCallToAction ActionArrowhttp://www.mayoclinic.org/news2010-sct/5937.html
  • InfoBoxCallToAction ActionArrowhttp://louisville.edu/medschool/pulmonary/diseases/interstitial-lung-disease-clinic/lymphan.html
  • InfoBoxCallToAction ActionArrowhttp://www.webmd.com/lung/lam-lung-disease-lymphangioleiomyomatosis
  • InfoBoxCallToAction ActionArrowhttp://www.nhlbi.nih.gov/health/dci/Diseases/lam/lam_treatments.html