Fresolimumab In Systemic Sclerosis

Inclusion Criteria:

• Meet ACR criteria for diffuse systemic sclerosis
• < 24 months since onset of first SSc manifestation other than Raynaud's phenomenon
• Modified Rodnan Skin Score ≥ 15
• Male or female adult ( ≥ 18 years of age)
• Able and willing to give written informed consent and comply with study protocol

Exclusion Criteria:

• Moderate or severe pulmonary disease w/ FVC < 80% or DLCO < 70% or ground glass and fibrosis > 20% of lung fields by HRCT
• Treatment with investigational drug within 4 weeks of screening
• Ongoing use of high dose steroids (> 10mg/day) or unstable steroid dose in past 4 weeks
• Treatment with immunosuppressive, cytotoxic, or antifibrotic drug within 4 weeks of screening
• Positive for HIV, HBV, and/or HCV
• Known active infection (bacterial, viral, fungal, mycobacterial, or other); not including fungal infection of nail beds or any major infection requiring hospitalization or treatment with IV antibiotics within 4 weeks of screening
• Patients w/ history of malignancy or premalignant lesion
• History of keratoacanthoma or squamous cell carcinoma
• Moderate to severe hepatic impairment
• SSc renal crisis within 6 months or creatinine > 2.0
• Lack of IV access for medication administration
• Moderate or severe cardiac disease with significant arrhythmia, heart failure, or unstable angina
• Anemia (Hb < 8.5 gm/dL)
• Thrombocytopenia or blood clotting disorder
• Patients with organ transplant (including allogeneic bone marrow transplant)