Lymphangioleiomyomatosis
OVERVIEW
Characterized by proliferation of atypical smooth muscle cells throughout the chest leading to destruction and infiltration of the lung parenchyma, cystic emphysema, and progressive deterioration of lung function.1-3
Low-grade, destructive, metastasizing neoplasm that spreads through the lymphatic system.1
EPIDEMIOLOGY
- Rare, affects less than 1 in 1 million people2,3
- Occurs exclusively in women aged 20-40 years2,3
- Prognosis is uncertain because clinical course is variable1,3
- Median survival is likely >8 years from diagnosis
- Progression may accelerate during pregnancy
DISEASE COURSE2,3
- Slowly progressive and over years often leads to respiratory failure and death
- Lung function declines 2-3x faster than in a healthy person
