Efficacy and Safety of Sirolimus in LAM

Inclusion Criteria: * Age 18 or older * Signed and dated informed consent * Diagnosis of LAM based on compatible chest CT scan and a) biopsy or cytology consistent with LAM, or b) presence of tuberous sclerosis, angiomyolipoma or chylous pleural effusion; or c) a VEGF-D level of at least 800 pg/ml * Forced expiratory volume in one second (FEV1) of 70% or less of predicted value after administration of a bronchodilator Exclusion Criteria: * Known allergy to sirolimus * History of heart attack, angina, or stroke due to clogging, narrowing, and hardening of the arteries and blood vessels * Significant hematologic or hepatic abnormality (transaminase levels greater than three times the upper limit of normal, HCT less than 30%, platelets less than 80,000/cubic mm, adjusted absolute neutrophil count less than 1,000/cubic mm, total white blood cell count less than 3,000/cubic mm) * Intercurrent infection at the time treatment with sirolimus begins * Any surgery involving entry into a body cavity or requiring three or more sutures within 8 weeks of initiation of study drug * Use of an investigational drug within the 30 days prior to random assignment * Uncontrolled hyperlipidemia * Previous lung transplant or currently on lung transplant list * Unable to attend scheduled study visits * Unable to perform pulmonary function tests * Creatinine levels greater than 2.5 mg/dl * Chylous ascites severe enough to affect diaphragmatic function * Pleural effusion severe enough to affect pulm…