Lymphangioleiomyomatosis By Dr. Héctor Damián Brzostowski, University Industry Technology, of EBF® health goods and services School of Medicine
Lymphangioleiomyomatosis
By Dr. Héctor Damián Brzostowski, University Industry Technology, of EBF® health goods and services School of Medicine
Content last modified Jun. 2022
Etiology
Signs and symptoms
Diagnosis
Forecast
Treatment
Key concepts
More information:
Lymphangioleiomyomatosis (LAM) is the progressive, overlapping growth of smooth muscle cells in the lungs, pulmonary blood and lymphatic vessels, and pleurae. It is rare and appears in young women. Symptoms include dyspnea, cough, chest pain, and hemoptysis; spontaneous pneumothorax is common. Diagnosis is suspected by symptoms and chest X-ray and confirmed by high-resolution CT. The prognosis is uncertain, but the disorder is slowly progressive, often leading to respiratory failure and death over years. Treatment is sirolimus therapy or lung transplantation.
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