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)