IVIG Treatment in Systemic Sclerosis

The investigators propose a double-blind controlled trial of 24 patients with Privigen®
(Immune Globulin Intravenous (Human), 10% liquid treatment) with 3:1 randomization. Subjects
with scleroderma will be given 2 g/kg/mo of Privigen® or placebo (Albuminar®-5) for 6 months.
Scleroderma is generally a progressive disease, and while the skin does improve with time in
some patients, others have progressive disease in spite of aggressive treatment. Also,
spontaneous improvement in other organ systems is even less likely. Patients entered into the
trial will have failed to respond to standard of care treatment over the past 4 months. Thus,
the investigators feel that any actual improvement observed can be attributed to the IVIG
treatment. Since this is a pilot study, future larger controlled trials will be necessary to
clearly demonstrate the effectiveness, but the investigators are hoping that this study will
give us signals that will guide the future clinical trial.

Inclusion Criteria:

- Diffuse systemic sclerosis with active skin involvement as defined as a mRSS >12, with
no improvement or worsening in the previous 4 months in spite of treatment with
methotrexate, cellcept, imuran, or anti-TNF agent;

- 18 years of age or older;

- Disease duration of less than 5 years from the first non-Raynaud's symptom of
scleroderma.

Exclusion Criteria:

- Use of more than 10 mg of prednisone, or any dose of cytoxan, d-penicillamine, or
rituximab in the last 3 months;

- History of deep vein thrombosis (DVT), stroke, or other thromboembolic phenomenon;

- History of anaphylaxis or other serious reaction to human blood or blood products.

- Absolute IgA deficiency

- A prior receipt of IVIg treatment