Evaluating the Effectiveness of Intravenous Immunoglobulin Therapy in Autoimmune Autonomic Ganglionopathy

Exclusion Criteria

• •1. Women of childbearing potential (WOCP) who are not using a medically accepted contraception
• •2. Pregnant or lactating females- if participants become pregnant during the trial they will no longer receive IVIG, but will be followed as part of the intention to treat protocol.
• •3. Severe depression and/or anxiety (score of \> 29 on the Beck Depression Inventory or score on the Beck Anxiety Inventory of ≥ 36)
• •4. Active psychosis is ineligible, history of psychosis will be eligible, but only after review with the patients PCP and/or treating mental health provider.
• •5. History of asthma
• •6. Other causes of autonomic failure (e.g., diabetes, amyloidosis)
• •7. History of allergic or anaphylactic reaction to humanized or murine antibodies.
• •8. History or presence of recurrent or chronic infection (recurrent infections defined as \>4 times per year).
• •9. History of cancer, including solid tumors and hematologic malignancies (except fully resolved and resected cutaneous basal cell and squamous cell carcinomas of the skin)
• •10. History or presence of vascular disease potentially affecting brain or spinal cord (e.g., stroke, transient ischemic attack, carotid stenosis (greater than 80%), aortic aneurysm, intracranial aneurysm, hemorrhage, arteriovenous malformation)
• •11. History of severe, clinically significant central nervous system trauma (e.g., cerebral contusion, spinal cord compression)
• •12. History or presence of infectious causes of encephalopathy or myelopathy (e.g., syphilis, Lyme disease, human T-cell lymphotropic virus type 1 \[HTLV-1\], herpes zoster myelopathy)
• •13. History of thromboembolic events or deep vein thrombosis
• •14. Platelet count \<100,000/mL, Hemoglobin \<8.5 g/dL, Neutrophils \<1.5 x 103/mL.
• •15. Serum IgA deficiency: Immunoglobulin A (IgA) level \< 7 mg/dL.
• •16. History of immunosuppression or HIV/AIDS
• •17. History of cardiac arrhythmia or angina, electrocardiogram (ECG) showing significant abnormality that the treating investigator determines may jeopardize the participant's health (i.e., acute ischemia, left bundle branch, or bifascicular block)
• •18. History of renal failure or creatinine \>2.0
• •19. History of previous allergic response to albumin.
• •20. Treatment with IVIG or plasma exchange within 6 weeks of study enrollment.
• •21. Active adjustments of other immunomodulatory treatments. Patients that are on stable doses of immunomodulatory medications (no dose changes within 4 months -including, but not limited to prednisone, mycophenolate mofetil or azathioprine) but still have elevated antibody titers and meet criteria for inclusion will be allowed to participate in the study.