Double-blind Placebo-controlled Pilot Study of Sirolimus in Idiopathic Pulmonary Fibrosis (IPF)

Inclusion criteria

• Male and female patients 21-85 years of age
• Individuals diagnosed with IPF, based on:

• clinical symptoms consistent with idiopathic pulmonary fibrosis (IPF) of > 3 months duration, plus
• histologically diagnosed UIP or diagnostic chest high resolution CT features of UIP, plus
• negative workup for known causes of UIP
• Ability to understand a written informed consent form and comply with the requirements of the study.

Exclusion criteria

• Clinical features or known diagnosis of an active infection, including untreated latent tuberculosis
• Clinical features or known diagnosis of malignancy
• Known diagnosis of an interstitial lung disease other than IPF including but not limited to sarcoidosis, hypersensitivity pneumonitis, non-specific interstitial pneumonia (NSIP).
• History of clinically significant environmental exposures known to cause interstitial lung disease (including but not limited to drugs, asbestos, silica, beryllium, radiation, domestic birds, etc).
• Diagnosis of any connective tissue disease (including but not limited to scleroderma, SLE, rheumatoid arthritis) or vasculitides according to the American College of Rheumatology criteria.
• Systolic blood pressure < 100 or >145 mm Hg or diastolic blood pressure < 50 or >90 mmHg
• Evidence of active infection within 1 week prior to enrollment.
• Recently started (<8 weeks prior to baseline visit) or planned cardiopulmonary rehabilitation program before conclusion of the study
• History of unstable or deteriorating cardiac disease, including but not limited to: myocardial infarction, coronary artery bypass surgery or angioplasty within the past 6 months, congestive heart failure requiring hospitalization within the past 6 months, or uncontrolled arrhythmia
• History of unstable or deteriorating neurologic disease, including but not limited to: TIAs or stroke
• Pregnant or lactating females. Females of child bearing potential are required to have a negative serum or urine pregnancy test prior to treatment and agree to practice abstinence or prevent pregnancy by at least a barrier method of birth control.
• Liver panel above specific limits at screening: Total bilirubin >1.5-fold upper limit of normal, AST, ALT or alkaline phosphatase > 3-fold upper limit of normal at screening.
• Hematology outside of specified limits, WBC <2,500/ mm3, hematocrit <30, platelets <100,000/mm3 at screening.
• Investigational therapy for any indication within 28 days prior to treatment.
• Current treatment with drugs that are strong inhibitors of CYP3A4 or P-gp, namely bromocriptine, cimetidine, cisapride, clotrimazole, danazol, diltiazem, fluconazole, HIV-protease inhibitors (e.g., ritonavir, indinavir), metoclopramide, nicardipine, troleandomycin, verapamil
• Inability or unwillingness to comply with the requirements for the trial.