Study of Pomalidomide (CC-4047) to Evaluate Safety, Tolerability, Pharmacokinetics, Pharmacodynamics and Effectiveness for Subjects With Systemic Sclerosis With Interstitial Lung Disease

Inclusion Criteria:

• Male or females between 18 and 80 years of age (inclusive) at the time of consent
• Diagnosis of SSC as defined by ACR criteria
• Onset of the first non-Raynaud's manifestation of SSC within 7 years of Screening
• Subjects are required to meet at least one of the following 2 pulmonary-related criteria to be eligible for the study:
• Repeat FVC at Baseline (Visit 2) within 5% of the FVC measured at Screening
• Carbon monoxide diffusing capacity (DLco) ≥ 35% and ≤ 80% of predicted value at Screening
• Abnormalities on high resolution computed tomography consistent with sclerodermatous involvement of the lung (eg, ground glass, honeycombing)

FVC ≥ 45% and <70% at Screening and Baseline (Visit 2) [with or without a documented pre-specified FVC decline or fibrosis score]

OR

FVC readings ≥ 70% and ≤ 80% at Screening and Baseline (Visit 2) with a documented history of either or both of:

1. A ≥ 5% decrease (expressed as percent predicted or in liters) in FVC in the 24-month period prior to Baseline (Visit 2) based on 3 or more assessments. Two assessments may be done during the Screening phase provided the assessments are completed at least 2 weeks apart.
2. An HRCT fibrosis score > 20%

Exclusion Criteria:

• Oxygen saturation (SpO2) < 92% (room air [sea level] at rest) at Screening or Baseline
• Known diagnosis of obstructive lung disease as defined by forced expiratory volume (FEV1)/FVC ratio < 0.7
• Diagnosis of pulmonary arterial hypertension (PAH) requiring treatment
• Known diagnosis of other significant respiratory disorders (e.g., asthma, tuberculosis, sarcoidosis, aspergillosis, chronic bronchitis, neoplastic disease, cystic fibrosis, etc.)
• Current clinical diagnosis of another inflammatory connective tissue disease (e.g., systemic lupus erythematosus, rheumatoid arthritis, Sjogren's syndrome, etc.)
• Use of melphalan within 52 weeks of Screening
• Additional concomitant medications which prolong the QT/QTc interval (measure of heart's electrical cycle) during the course of the study
• Use of any anti-coagulant or anti-thrombotic medications (other than low dose-aspirin [(≤ 100 mg/day)
• Use of any cytotoxic/immunosuppressive agent (other than prednisone ≤ 10 mg/day [mean dose] or equivalent), including but not limited to azathioprine, cyclophosphamide, methotrexate, mycophenolate and cyclosporine within 28 days (4 weeks) of Screening
• Use of any biologic agent within 84 days (12 weeks) or 5 half-lives of Screening. In the case of rituximab, use within 168 days (24 weeks) of Screening or no recovery of CD20-positive B lymphocytes if the last dose of rituximab has been more than 24 weeks prior to Screening
• Use of bosentan, ambrisentan, sildenafil, tadalafil for PAH within 28 days (4 weeks) of Screening
• Use of medications (e.g., D-penicillamine, Potaba) with putative scleroderma disease-modifying properties within 4 weeks of Screening
• Use of any investigational drug within 4 weeks of Screening or 5 pharmacodynamic/pharmacokinetic half-lives if known (whichever is longer)
• Smoking of cigars, pipes or cigarettes within 24 weeks of Screening