Lymphangioleiomyomatosis (LAM) is a rare, cystic lung disease that is associated with
mutations in tuberous sclerosis genes, renal angiomyolipomas, lymphatic spread, and
remarkable female gender restriction. The clinical course of LAM is characterized by
progressive dyspnea on exertion, recurrent pneumothorax, and chylous fluid collections.
Lung function declines at approximately twofold to threefold times the rate of healthy
subjects, based on an annual drop in FEV 1 of 75 to 120 mL in reported series. The …