Understanding Lymphangioleiomyomatosis: Causes, Symptoms, and Management

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Lymphangioleiomyomatosis

by Alike Medical Team ∙ Updated on June 13, 2023

General

Lymphangioleiomyomatosis (LAM) is a genetic lung disease characterized by an abnormal growth of smooth muscle cells, which causes holes or cysts formation in the lung. It is typically diagnosed in women between the ages 20-40, 30% of them suffer from tuberous sclerosis. In some cases the disease is hereditary and there is also a non hereditary form. People who have this condition suffer from breathing difficulties as the gas exchange becomes less effective and have greater risk for developing pneumothorax. Furthermore, they have a tendency to develop angiolipomas, which are growth in the kidneys, which are harmless unless they become enlarged and cause bleeding.

3 people with Lymphangioleiomyomatosis

Learn from others who are experiencing Lymphangioleiomyomatosis.

Signs & symptoms

Symptoms may include shortness of breath, chest pain, cough, wheezing, pneumothorax, and pleural effusion.

Diagnosis

Diagnosis is based upon findings from medical history and physical examination. Further evaluation may include chest CT scan, lung function tests, VEGF-D blood tests, to measure the level of this hormone in the blood, which is elevated in LAM and a lung biopsy.

Treatment

There is no cure for lymphangioleiomyomatosis but there is a treatment to prevent it from getting worse which is called Sirolimus. Other treatments include oxygen therapy, inhaled medications, procedures to remove fluid from the lungs and lung transplant in severe cases.

Note

☝ We provide information on prescription and over-the-counter medicines, diagnosis, procedures and lab tests. This material is provided for educational purposes only and is not medical advice, diagnosis or treatment.

National Institutes of Health ∙ World Health Organization ∙ MedlinePluse ∙ Centers for Disease Control and Prevention

☝ All information has been reviewed by certified physicians from Alike

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