Signs, Symptoms, and Complications

The uncontrolled growth of LAM cells and their effect on nearby body tissues causes the signs, symptoms, and complications of LAM. Symptoms tend to start when women are between the ages of 20 and 40.

Usually, TSC-LAM is milder than sporadic LAM and may not cause symptoms affecting the lungs. However, the severity of the disease varies from patient to patient and it is still possible for some women with sporadic LAM to also have mild disease without symptoms and some women with TSC-LAM to have more severe disease with worse symptoms and complications.

Signs

Signs of LAM are:

  • Lung cysts detected by chest imaging tests
  • Increased VEGF-D levels in the blood. VEGF-D is a vascular growth factor involved in tumor spread.
  • Reduced lung function
  • Reduced oxygen levels in the blood

Symptoms

The most common symptoms are:

  • Chest pain or aches that may worsen when you breathe in.
  • Fatigue that may affect your overall quality of life.
  • Frequent cough that may occur with bloody phlegm.
  • Shortness of breath that at first may occur only during high-energy activities but over time may happen after simple activities such as dressing and showering.
  • Wheezing or a whistling sound when you breathe.

Complications

Possible complications of LAM include any of the following:

  • Angiomyolipomas and other tumors. Many women who have LAM get tumors in their kidneys, called angiomyolipomas. Women who have LAM also may develop large tumors in the lymph node or growths in other organs such as the liver.
  • Blood in the urine. This may occur in women who have kidney tumors.
  • Enlarged lymph nodes. These usually occur in the abdomen or the chest. Very rarely, enlarged lymph nodes may occur in locations where they can be felt, such as the neck or under the arms.
  • Pleural effusions. This condition can occur if bodily fluids collect in the space between the lung and the chest wall. Often the fluid contains a milky substance called chylothorax. The excess fluid in the chest may cause shortness of breath because the lung has less room to expand.
  • Pneumothorax or collapsed lung. This potentially life-threatening condition occurs when air leaks out of the lung and into the space between the lung and chest wall, an area called the pleural space. In LAM, a pneumothorax can occur if lung cysts rupture through the lining of a lung. Air that collects in the space between the lung and chest wall must be removed to reinflate the lung. A collapsed lung can cause pain and shortness of breath. Sometimes one lung will collapse repeatedly. Pneumothorax usually requires urgent medical care and treatment.
  • Swelling or the build-up of fluid. This can happen in the abdomen, pelvic area, legs, ankles or feet. Pain may also occur with the swelling.

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What causes the signs, symptoms and complications of LAM?

When a person has LAM, abnormal muscle-like cells begin to grow out of control in certain organs or tissues, especially the lungs, lymph nodes, and kidneys. Over time, these LAM cells can destroy the normal lung tissue. As a result, air no longer moves freely in and out of the lungs. In some cases, this means the lungs can’t supply enough oxygen to the body’s other organs.

Normal Lungs and Lungs With LAM. Figure A shows the location of the lungs and airways in the body. The inset image shows a cross-section of a healthy lung. Figure B shows a view of the lungs with LAM and a collapsed lung (pneumothorax). The inset image shows a cross-section of a lung with LAM.