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Lymphangioleiomyomatosis

 

The lymphangioleiomyomatosis is one belonging to the category of diseases Interstitial lung disease. Under this denomination processes of different origin are grouped, in which the walls of the alveoli and the perialveolar tissue are affected. They are not malignant diseases and are not caused by defined infectious agents.

The onset is insidious, not acute, and the diseases are often chronic. The initial response is inflammation of the air spaces and alveolar walls, which would cause an acute form of intraluminal and mural alveolitis (that is, both the lumen and the walls of the alveoli would be affected).

If the disease is chronic, the inflammation spreads to the interstitium and adjacent vessels, causing interstitial fibrosis.

Causes of lymphangioleiomyomatosis

The origin of this disease is not well known. There is a proliferation of smooth muscle in lymphatic vessels, bronchioles and pulmonary vessels.

It most frequently affects women of childbearing age; the fact that the disease worsens during pregnancy, postpartum and after estrogen treatment seems to indicate that there is an association between this disorder and the secretion of estrogens (female sex hormones).

Immature smooth muscle cells can proliferate in and around the lung tissue around the bronchial, vascular, and lymphatic structures, causing local obstruction and creating constrictive lesions, which turn into cysts. Vessels and lymph nodes of other organs can also be affected.

Symptoms of lymphangioleiomyomatosis

The most frequent clinical manifestations are:

  • Cough.
  • Dyspnoea.
  • Hemoptysis, due to obstruction of the pulmonary vessels.
  • Pneumothorax , due to the formation of pulmonary cysts.
  • Chylous effusion, due to obstruction of the lymphatic vessels.

Reticulonodular images (areas that resemble intersecting webs and nodular-like areas) and small cystic areas or diffuse honeycomb pattern in the lung fields appear on chest radiographs.

The CT scan shows images of thin-walled cysts with reticulonodular infiltrates.

Diagnosis and treatment

The clinic and radiological findings can help to make the diagnosis, but this is definitively established with the transbronchial or open lung biopsy.

Treatment is medroxyprogesterone acetate, intramuscularly for one year. If there is no improvement in this time interval, surgery called bilateral oophorectomy (removal of both ovaries) is recommended.

Treatment of complications that may appear, such as pneumothorax and chylous effusion, is necessary. Chemical or surgical pleurodesis would be performed.

In the advanced stages, lung transplantation is another alternative.

The evolution is towards a picture of respiratory failure in ten years.

How can I avoid it?

As it is a disease of unknown origin, it is not possible to take any measure to prevent its appearance.

At the onset of respiratory symptoms, such as cough, dyspnea or hemoptysis, the doctor must make a differential diagnosis with other pulmonary processes with similar manifestations.

If you have episodes of hemoptysis (expectoration of red blood in variable amounts with coughing fits) or symptoms of pneumothorax (chest pain with a sudden onset that increases with deep inspiration and is accompanied by a sensation of shortness of breath), you should go to your doctor.

 

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Hello Readers, I am Nikki Bella a Psychology student. I have always been concerned about human behavior and the mental processes that lead us to act and think the way we do. My collaboration as an editor in the psychology area of ​​Well Being Pole has allowed me to investigate further and expand my knowledge in the field of mental health; I have also acquired great knowledge about physical health and well-being, two fundamental bases that are directly related and are part of all mental health.

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