Lymphangioleiomyomatosis is a rare lung disease that affects the lungs of women of childbearing age. In people affected with Lymphangioleiomyomatosis, the cells that line the airways of the lungs and blood vessels begin to multiply rapidly. Due to this rapid multiplication, these cells accumulate in areas where they do not belong. Symptoms are:
• Shortness of breath
• Cough, with blood sometimes
• Sudden collapsed lung
• Chest pain
HOW IS LYMPHANGIOLEIOMYOMATOSIS DIAGNOSED?
Pulmonologists diagnose Lymphangioleiomyomatosis based on your symptoms and physical examination. Common diagnostic tests are chest X-ray, pulmonary function tests and CT-scan of lungs. For conducting lung biopsy, bronchoscopy, open lung biopsy and thoracoscopy are conducted
HOW IS LYMPHANGIOLEIOMYOMATOSIS TREATED?
The drug sirolimus (Rapamune) is the first drug approved to treat lymphangioleiomyomatosis. Hormone based treatments are also found to be effective. If pulmonary lymphangioleiomyomatosis progresses and becomes disabling, lung transplantation may be an option.
DID YOU KNOW?
This rare lung disease mostly affects women of childbearing age.