Lymphangioleiomyomatosis (cont.)

The survival time following diagnosis is uncertain, as the disease seems to be highly individual. It had been reported to be less than 10 years, but new reports show patients living more than 20 years after diagnosis.

Diagnosis:The diagnosis of LAM can be difficult because many of the early symptoms are similar to those of other lung diseases, such as asthma, emphysema, or bronchitis. Often the person first goes to the physician complaining of chest pain and shortness of breath that was caused by a pneumothorax. Some patients first consult their physician because of shortness of breath upon exertion. There are a number of tests the physician can do to gather information about LAM:

  • Chest X-ray -- This picture of the lungs and other tissue in the chest is used to diagnose a pneumothorax or the presence of fluid in the chest cavity (pleural effusion). Smooth muscle cysts, consistent with LAM, do not usually appear on X-ray.
  • Pulmonary function tests -- The patient breathes through a mouthpiece into a machine (spirometer) that measures the volume of air in the lungs, the movement of air into and out of the lungs, and the movement of oxygen from the lungs into the blood.
  • Blood test -- Blood is analyzed to determine whether the lungs are providing an adequate supply of oxygen to the blood.
  • Computed tomography (CT) -- The most definitive imaging test for diagnosing LAM. On a CT scan, the presence of thin-walled cysts spread relatively uniformly throughout the lungs usually means LAM. An abdominal CT scan may disclose a benign kidney tumor called angiomyolipoma that is associated with LAM.
  • Lung biopsy -- Two or more of the characteristic findings of LAM (cysts, fluid in the lungs, benign kidney tumor, and collapsed lung) may be considered diagnostic of LAM. An open lung biopsy should be performed as a last resort to diagnose LAM. In this procedure, a few small pieces of lung tissue are removed through an incision made in the chest wall between the ribs. Another procedure, thoracoscopy, is also being used in some patients to obtain lung tissue. In this procedure, tiny incisions are made in the chest wall, and a small lighted tube (endoscope) is inserted so that the interior of the lung can be viewed, and small pieces of tissue are removed. Both of these biopsy procedures must be done in the hospital under general anesthesia.

    Another technique, called 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. Bits of lung tissue are sampled, using a tiny forceps. This procedure is usually done in a hospital on an outpatient basis under local anesthesia. It is less reliable than an open lung biopsy because the amount of tissue that can be sampled is sometimes inadequate for diagnostic studies. After the lung tissue is removed, it is examined in a pathology laboratory for the presence of the abnormal muscle cells and cystic changes characteristic of LAM.

Treatment: Because LAM affects almost exclusively women of childbearing age, it has been thought that the hormone estrogen might be involved in the abnormal muscle cell growth that characterizes the disease, just as it is in the growth of smooth muscle in the uterus in a woman's childbearing years. Although there is no firm evidence that there is a relationship between estrogen and LAM, the treatment of ther disease has focused on reducing the production or effects of estrogen. The response to treatment has been highly individual, and no therapy has been found to be effective for all LAM patients. Oxygen therapy may be necessary if the disease continues to worsen and lung function is impaired.

For LAM patients with severe disease, lung transplantation is an established therapy. One year survival following transplant is approximately 70 percent, and 3-year survival is approximately 50 percent.

Effect on lifestyle: In the early stages of the disease, most patients can go about their daily activities, including attending school, going to work, and performing common physical activities, such as walking up a hill. In more advanced stages, the patient may have very limited ability to move around and may require oxygen full-time. Patients with LAM should follow the same healthy lifestyle recommended for the general population, including eating a healthy diet, getting as much exercise as they can, as well as plenty of rest, and, of course, not smoking. Traveling to remote areas where medical attention is not readily available or to high altitudes where the blebs can expand and rupture should be considered carefully before undertaken.

In patients with normal lung function, there is probably no increased risk associated with pregnancy. However, in patients with compromised lung function, pregnancy is not advised. There do not appear to be complications associated with oral contraceptives, but this issue should be discussed with the patient's pulmonologist and gynecologist.

Last Editorial Review: 5/13/2016

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