Fresolimumab (Anti-TGF-β)

IV Study Medication w/ Skin Biopsies for Diffuse Scleroderma

Inclusion:

• Must meet the American College of Rheumatology criteria for systemic sclerosis with diffuse cutaneous involvement and <24 months since the onset of the first SSc manifestation other than Raynaud’s phenomenon
• Must have a MRSS of ≥ 15
• Male or female ≥18 years of age
• Able and willing to give written informed consent and comply with the requirements of the study protocol

Exclusion:

• Treatment with any investigational agent within 4 weeks of screening or 5 half-lives of the investigational drug (whichever is longer)
• Ongoing use of high dose steroids (>10mg/day) or unstable steroid dose in the past 4 weeks
• Treatment with immunosuppressive (other than low dose steroids), cytotoxic or antifibrotic drug within 4 weeks of screening
• Known reactive for HIV
• Positive viral hepatitis B or hepatitis C serologies
• History of malignancy or lesion considered premalignant
• Prior history of keratoacanthoma or squamous cell carcinoma
• Scleroderma renal crisis within 6 months or creatinine greater than 2.0
• Pregnancy (a negative pregnancy test will be performed for all women of childbearing potential on the day of treatment)
• Moderately severe pulmonary disease with FVC <80%, or DLCO <70% predicted, or ground glass and fibrosis involving greater than 20% of the lung fields by HRCT
• Moderately severe cardiac disease with either a history of significant arrhythmia
• Organ transplant, including those that have received an allogeneic bone marrow transplant
• Lack of intravenous access for medication administration