IVIG (Gamunex-C) Treatment Study for POTS Subjects

Inclusion Criteria

• 18 years of age or older, and able to provide informed consent
• Diagnosis of POTS (see Table 1)
• COMPASS-31 symptom score showing moderate to severe autonomic symptoms
• At least 3 of the following clinical or laboratory features of autoimmunity
• One or more serum autoantibodies (ANA ≥ 1:160, gAChR antibody > 0.2 nmol/L, positive ENA, aPL, TTG, gliadin) or inflammatory markers (ESR > 30, CRP > 2, low C3 complement or low immunoglobulin IgG level)
• Confirmed personal history or family history of defined autoimmune disease including Hashimoto's thyroiditis, celiac disease, antiphospholipid syndrome, rheumatoid arthritis, SLE, or Sjogren's syndrome
• Clear history of acute or subacute onset following infection, immunization, injury/concussion, surgery or pregnancy.
• Evidence of esophageal, gastric or intestinal dysmotility (with weight loss)
• Evidence of small fiber neuropathy (abnormal QSART or IENFD)
• Stable oral medical therapy for past 3 months
• Ambulatory at time of screening

Exclusion Criteria

• Current or previous immunosuppression therapy or IVIG treatment
• Contraindication to intravenous immunoglobulin or intravenous albumin
• Known allergic reactions to blood products including intravenous immunoglobulin (IVIG) and/or subcutaneous immunoglobulin (SCIG), such as history of clinically relevant hemolysis after IVIG infusion, aseptic meningitis, recurrent severe headache, hypersensitivity, severe generalized or severe local skin reaction.
• Inadequate peripheral venous access
• Evidence of renal insufficiency (Cr > 1.5 x elevated) or liver disease (transaminases > 2.5x upper limit) at screening
• History of thrombotic episode within 3 years of enrollment
• Other major medical issue which, in investigators opinion, increases risk for adverse event over the next 12 months or may require separate management.
• Female patients who are premenopausal and are (a) pregnant based on serum pregnancy test, or (b) breast-feeding.