LYMPHANGIOLEIOMYOMATOSIS

Lymphangioleiomyomatosis (LAM) is a lung disease characterized by the abnormal development of smooth muscle cells, particularly in the lungs and lymphatic systems. This abnormal growth causes holes or cysts to emerge in the lungs.

People with LAM have problems breathing because it is more challenging to move air in and out of the bronchial passages. Moreover, the replacement of normal lung tissue with holes or cysts weakens the capacity of the lungs to circulate oxygen into the blood.

SYMPTOMS

Patients with lymphangioleiomyomatosis (LAM) may experience the following symptoms due to abnormal growth of smooth muscle cells and cysts in their lungs:

  • Breathing problems
  • Chest pain
  • Coughing with phlegm or blood streaks
  • Wheezing
  • Pleural effusions (a fluid that accumulates in the chest cavity in which the lung sits).
  • Pneumothorax (collapsed lung).

DIAGNOSIS

Because the symptoms of lymphangioleiomyomatosis (LAM) are comparable to those of asthma or bronchitis, most women with the condition are unaware of it or are given the wrong diagnosis by clinicians who are unfamiliar with it.

So, the doctor will check you and ask about your symptoms in order to diagnose LAM. Certain tests, such as the ones mentioned below, may be ordered by the doctor:

  • Computed tomography (CT) scan. This scan generates a clear image of the lungs to detect whether cysts, the most common symptom of the condition, are present.
  • Lung function tests. This test involves breathing into a spirometer to assess how much air you can inhale and expel and if your lungs are working correctly.
  • Pulse oximetry. This test measures the amount of oxygen in your blood using a little gadget linked to your finger.
  • VEGF-D blood test. This blood test determines the amount of VEGF-D in your body. 
  • Abdominal CT scan or MRI. These scans provide a picture of the abdomen and may aid in diagnosing LAM.
  • Lung biopsy. In rare situations, a small piece of lung tissue must be obtained and examined under a microscope to diagnose LAM or rule out other disorders.

TREATMENT

Although there is no cure for lymphangioleiomyomatosis (LAM), there is now a treatment that can help stabilize the disease and keep it from worsening. Patients who show signs of lung function loss due to LAM are given the drug sirolimus. Everolimus, a medication from the same class, can also be used to treat LAM in some cases.

Other lymphangioleiomyomatosis therapies that may be employed in certain circumstances include:

  • Oxygen therapy
  • Inhaled medications
  • Lung transplant

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