Lymphangioleiomyomatosis – Causes, Symptoms and Treatments!

Lymphangioleiomyomatosis – Causes, Symptoms and Treatments  that many do not know. Furthermore, Lymphangioleiomyomatosis is a complex and rare lung disease that usually affects women of childbearing age. Lymphangioleiomyomatosis  triggers  the body to make an abnormal amount of muscle cells that deposit in the body’s organs. Most commonly, these cells accumulate in the kidneys , lungs , and lymph nodes.

As these cells do not play the role of the organ they collected from, the organ will not function efficiently and can cause serious complications for the patient. Lymphangioleiomyomatosis in the lungs causes a decrease in the amount of oxygen that can be transferred to the body, resulting in hypoxia or reduced delivery of oxygen to the organs.

Causes of Lymphangioleiomyomatosis:  There are two types of Lymphangioleiomyomatosis , but both have an unknown cause. Most cases occur in women between the ages of 20 and 40, and there is a higher occurrence in women who have a condition known as tuberous sclerosis complex (TSC). About 30% of women with TSC will develop Lymphangioleiomyomatosis , but it is usually a milder form compared to women who develop the condition spontaneously.

Symptoms of Lymphangioleiomyomatosis: The uncontrolled growth of Lymphangioleiomyomatosis , it’s cells and their effect on nearby body tissues causes the signs and symptoms of Lymphangioleiomyomatosis . The most common signs and symptoms are:

  • Shortness of breath: Especially during physical activity. First, shortness of breath can only occur during high-energy activities. Over time, you may have trouble breathing even during simple activities like dressing and showering.
  • Chest Pain or Aches: This pain can be worse when you breathe.
  • Frequent Cough: This can occur with bloody phlegm.
  • wheezing.

Other signs and symptoms of Lymphangioleiomyomatosis include

  • Pneumothorax : Or collapsed lung . This condition can occur if lung cysts break through the lining of a lung . Air that accumulates in the space between the lung and the chest wall must be removed to re-flare the lung .
  • Pleural Effusions: This condition can occur if body fluids accumulate in the space between the lung and the chest wall. Often, the fluid contains a milky substance called chyle. Excess fluid in the chest can cause shortness of breath because the lung has less room to expand.
  • Blood in the Urine: This sign can occur in women who have kidney tumors called angiomyolipomas.
  • Enlarged Lymphatic Nodes: These usually occur in the abdomen or chest . Very rarely, enlarged lymph nodes can occur in places where they can be felt, such as the neck or under the arms.
  • Abdominal Swelling:  Sometimes with pain.
  • Other Body Swelling:  As in the legs , ankles or feet.

Other illnesses can also cause many of these signs and symptoms. It is important to see your doctor and find out what is causing these problems.

Diagnostic Test:  Providers will have to rule out other medical conditions such as asthma , bronchitis , and emphysema to provide a diagnosis of  Lymphangioleiomyomatosis . Patients can be treated by a pulmonologist, depending on the case. Using a combination of patient history, diagnosis and lung function testing is the common approach. Tests that can be used include:

• Pulmonary function studies.
• Chest X-ray.
• Pulse oximetry.
• High resolution computed tomography.
• Blood work.
• Lung biopsies .
• Thoracoscopy.

Lymphangioleiomyomatosis Treatment:  There is no treatment available to stop the growth of Lymphangioleiomyomatosis cells in the body. Treatment focuses on treating symptoms and preventing complications. Some approaches used to treat  Lymphangioleiomyomatosis include:

  • Medicines to open the lungs and move as much air as possible.
  • Oxygen therapy.
  • Procedures to remove fluid from the chest.
  • Lung transplant .
  • Procedures to shrink kidney tumors , if present.
  • Hormone therapy, if appropriate.

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