Lymphangioleimyomatosis (LAM)
LAM is characterized by an unusual type of muscle cell that invades the tissue of the lungs, including the airways, blood and lymph vessels. Over time, these muscles cells form into bundles (cysts or blebs) and grow into the walls of the airways, causing them to become obstructed thereby blocking the flow of air, blood, lymph (the fluid which is derived from connective tissue and tissue between organs) to and from the lungs. This blockage prevents the lungs from providing oxygen to the rest of the body.
This disorder also may affect the smooth muscle tissue of the thoracic duct, lymph nodes, the liver, and in very rare cases, the lymphatic vessels of the legs.
Symptoms
Shortness of breath (in early stages of LAM, shortness of breath occurs only during strenuous exercise. As the disease progresses there may be shortness of breath while resting)
The early symptoms of LAM are similar to those of other lung diseases such as asthma, or bronchitis. Therefore the doctor must run a battery of tests to accurately diagnose LAM. These tests include a chest x-ray, pulmonary function tests, blood tests, and a computed tomography (CT) scan. If these tests are inconclusive the doctor will perform a lung biopsy.
A biopsy is a diagnostic test in which tissue or cells are removed from the body for examination under the microscope. In the case of LAM, lung tissue can removed via at least three different procedures.
The first is open lung biopsy (tissue is removed through an incision made in the chest wall between the ribs).
The second is thoracoscopy (a small incision is made through which a small lighted tube called an endoscope is inserted). This instrument allows the doctor to view the lung and remove the tissue.
The third procedure is transbronchial biopsy (tissue is removed through a long, narrow flexible tube called a bronchoscope which is inserted down the windpipe and into the lungs).
After the lung tissue is removed, the laboratory will look for the presence of abnormal muscle cells and cystic changes.
Treatment
The aim of treatment is controlling the symptoms of the disease.
LAM is characterized by an unusual type of muscle cell that invades the tissue of the lungs, including the airways, blood and lymph vessels. Over time, these muscles cells form into bundles (cysts or blebs) and grow into the walls of the airways, causing them to become obstructed thereby blocking the flow of air, blood, lymph (the fluid which is derived from connective tissue and tissue between organs) to and from the lungs. This blockage prevents the lungs from providing oxygen to the rest of the body.
This disorder also may affect the smooth muscle tissue of the thoracic duct, lymph nodes, the liver, and in very rare cases, the lymphatic vessels of the legs.
Symptoms
Shortness of breath (in early stages of LAM, shortness of breath occurs only during strenuous exercise. As the disease progresses there may be shortness of breath while resting)
- Chest pain (due to excessive or air in the chest cavity.
- Coughing up small amounts of blood or blood-stained sputum
- Difficulty breathing
- Collapsed lung ( symptoms include sudden, sharp chest pain; difficult rapid breathing; rapid heart beat profuse sweating; dizziness; lack of normal chest movement)
- Weight loss
- Abdominal pain and discomfort (due to fluid collection in the walls of the abdomen)
- Cloudy urine
The early symptoms of LAM are similar to those of other lung diseases such as asthma, or bronchitis. Therefore the doctor must run a battery of tests to accurately diagnose LAM. These tests include a chest x-ray, pulmonary function tests, blood tests, and a computed tomography (CT) scan. If these tests are inconclusive the doctor will perform a lung biopsy.
A biopsy is a diagnostic test in which tissue or cells are removed from the body for examination under the microscope. In the case of LAM, lung tissue can removed via at least three different procedures.
The first is open lung biopsy (tissue is removed through an incision made in the chest wall between the ribs).
The second is thoracoscopy (a small incision is made through which a small lighted tube called an endoscope is inserted). This instrument allows the doctor to view the lung and remove the tissue.
The third procedure is transbronchial biopsy (tissue is removed through a long, narrow flexible tube called a bronchoscope which is inserted down the windpipe and into the lungs).
After the lung tissue is removed, the laboratory will look for the presence of abnormal muscle cells and cystic changes.
Treatment
The aim of treatment is controlling the symptoms of the disease.
- For persons with excess fluid in the lungs, there are at least two methods to reduce the fluid. The first method is the insertion of shunts (tubes) in the lungs. The other method is the surgical insertion of a sterile irritant, such as the antibiotic tetracycline, into the space surrounding the lungs. This method not only reduces the fluid, but also helps in reducing the size of the chest cavity.
- For minor breathing problems, the doctor may prescribe a bronchodilator drug that relaxes the muscles around the bronchioles and assists in widening the airways in the lungs. For major breathing problems, oxygen therapy may be necessary if the disease continues to worsen and lung function is impaired.
- In response to the theory that estrogen production may be a causative factor of LAM, doctors may opt too pharmaceutically or surgically reduce estrogen production.
- Removal of the abnormal lung tissue.
- Lung transplantation is considered as a last resort.