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Diagnosing LAM

Because many of the early signs and symptoms of lymphangioleiomyomatosis (LAM) are similar to those of other lung diseases, including asthma, emphysema and bronchitis, LAM can be difficult to diagnose. In fact, many women have LAM and don't know it.

Often a woman with LAM first goes to her physician complaining of chest pain and shortness of breath that was caused by a lung collapse.  Some patients first consult their physician because of shortness of breath with physical activity. There are a number of tests the physician can run to confirm or rule out the existence of LAM, assess the spread of the disease or determine the extent of lung damage: 

Chest X-ray - takes a picture of your heart, lungs and surrounding tissue. It can show whether you have a collapsed lung or a build up of fluid around the lungs. The X-ray may show cysts or clusters of cells on the lungs, which are suggestive of LAM; however, this is not the optimal way to diagnose the disease.

High-resolution CT scan - provides a more detailed (two-dimensional) image of the inside of your lungs and chest. It is the most accurate imaging test for diagnosing LAM. A computed tomography, or CT scan, can reveal cysts or abnormal clusters of cells in your lungs, a collapsed lung or enlarged lymph nodes. It can also show the extent to which the cysts have spread. An abdominal CT scan is also recommended, as benign kidney tumors, known as angiomyolipomas, are found in 40 percent of women with LAM.

Lung biopsy - involves removing samples of lung tissue, which are examined under a microscope to look for abnormalities that may indicate LAM. There are several ways physicians can remove lung tissue.

Thoracoscopy, also called video-assisted thorascopic surgery (VATS), is used to insert a small lighted tube (endoscope) into tiny incisions in your chest wall so that the interior of the lung can be viewed, and small pieces of tissue are removed.  This procedure must be done in the hospital under general anesthesia.

Open biopsy should only be performed as a last resort to diagnose LAM; recovery is longer than other, less invasive methods. In this procedure, a few small pieces of lung tissue are removed through an incision made in the chest wall between the ribs.  This procedure also takes place in the hospital under general anesthesia.

Transbronchial biopsy may also be used to obtain a small amount of lung tissue. A long, narrow, flexible, lighted tube (bronchoscope) is inserted down the windpipe (trachea) and into the lungs. Pieces of lung tissue are sampled using a tiny forceps. This procedure is usually done in a hospital on an outpatient basis under local anesthesia. However, the amount of tissue that can be sampled is usually not adequate to definitively diagnose LAM.

Lung function test - evaluates how well your lungs are working by measuring the amount (volume) of air inhaled or exhaled, and how much time each breath takes (rate). The patient breathes through a mouthpiece into a machine called a spirometer. The spirometer records the movement of air into and out of the lungs. Although these tests are used to determine the effect LAM has on lung function, they are not typically used for diagnosis.

Blood Tests - a sample of blood is analyzed to determine whether your lungs are providing enough oxygen to the bloodstream, but cannot diagnose LAM.

 

 

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