Inclusion Criteria:

   - Female patients. It should be noted that LAM occurs almost exclusively in women.

   - 18 to 65 years of age.

   - All patients must have a diagnosis of LAM as defined by compatible cystic changes on
   chest computed tomography (CT) and one of the following:

   - Open lung, transbronchial or thoracic needle biopsy consistent with LAM

   - Open or needle abdominal biopsy findings consistent with LAM

   - Clinical findings of tuberous scleroma complex (TSC), renal angiomyolipoma, cystic
   abdominal lymphangiomas, or history of chylous effusion in the chest or abdomen

   - Serum vascular endothelial growth factor D (VEGF-D) > 800 pg/ml

   - Subjects must have had a recent reduction in forced expiratory volume at 1-second
   (FEV1) of > 50ml/year, as shown by at least two pulmonary function testing (PFT)
   measured at least 6 months apart in the last 24 months prior to enrolling study.

Exclusion Criteria:

   - Current infection.

   - Major surgery within the past 2 months

   - Advanced hematologic, renal, hepatic, non-LAM lung disease or metabolic diseases; or
   BMI of >35

   - The use of another investigational drug within 30 days

   - The use of mTOR (mammalian target of rapamycin) inhibitors within 30 days

   - Previous lung transplantation.

   - Inability to attend scheduled clinic visits

   - Inability to give informed consent

   - Inability to perform pulmonary function testing

   - History of malignancy in the past two years, other than squamous or basal cell skin
   cancer or status post successful excision or treatment.

   - Nursing mothers

   - Current or planned pregnancy.

   - Not using adequate contraception (in woman of childbearing potential).

   - Significant clinical change in health in the past 30 days