pulmonary disorders
Lymphangioleiomyomatosis
Key Points
Lymphangioleiomyomatosis (LAM) can mimic interstitial lung disease but is actually a rare, slowly progressive growth of smooth muscle cells in various organs. Consider the diagnosis in young women with unexplained dyspnea plus interstitial changes with normal or increased lung volumes on chest x-ray, spontaneous pneumothorax, or chylous effusion. Do high-resolution CT and VEGF-D testing; if results are inconclusive, biopsy. Considersirolimustreatment for LAM with abnormal or progressively decreasing pulmonary function. 