Lymphangioleiomyomatosis

Excerpt

Lymphangioleiomyomatosis (LAM) is a primary disease of the lung parenchyma typified by abnormal growth of atypical smooth muscle cells in the lung vasculature, lymphatics, and alveoli that leads to the formation of multiple cysts in the lungs bilaterally causing respiratory symptoms, such as fatigue and dyspnea on exertion. The main involved organs are the lungs, but LAM can have extrapulmonary manifestations in the kidneys, causing benign renal angiomyolipomas, or can sometimes cause perivascular epithelioid cell tumors with visceral organ involvement. This article discusses the etiology, epidemiology, clinical manifestations, and management options of LAM.