COLA: A Pilot Clinical Trial of COX-2 Inhibition in LAM and TSC

Inclusion Criteria: * Female of age 18 to 69 * Ability to give informed consent * Definite diagnosis of LAM Typical cystic change on CT scan of the chest plus one of the following i) biopsy or cytology of any tissue demonstrating LAM, ii) angiomyolipoma, chylothorax, clinical or genetic diagnosis of tuberous sclerosis, iii) serum VEGF-D \> 800pg/ml * post-bronchodilator forced expiratory volume in one second ≥ 70% of predicted and DLCO ≥ 70% predicted during baseline visit. * Women of childbearing potential must agree to use two forms of barrier contraception after screening visit, for the duration of study participation and for 30 days after last dose. Exclusion Criteria: * History of intolerance to non-steroidal anti-inflammatory drugs (NSAIDs) * History of current regular use (daily most days of the week) of NSAIDs * History of use of rapamycin or everolimus * Uncontrolled intercurrent illness * Pregnant, breast feeding or planning to become pregnant in the next 2 years * Significant hematological (platelet count \<100.000/µl or hepatic abnormalities (Liver function tests \>2 times normal). * Use of an investigational drug within 30 days of study start * Inability to attend scheduled clinic visits * Inability to give informed consent * Inability to perform spirometry * Creatinine \> 1.0 mg/dl or eGFR \< 60 ml/min * Pneumothorax within past 8 weeks * History of malignancy in the last 2 years other than basal cell skin cancer * Use of estrogen containing medication within…