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Intravenous Immunoglobulin

as a Potential Treatment for Autoimmune Dysautonomia

Intravenous immunoglobulin therapy (IVIg) is FDA approved for immune-mediated peripheral nerve disorders including Guillain-Barre syndrome, chronic inflammatory demyelinating polyneuropathy, and multifocal motor neuropathy. While not yet FDA approved, immunoglobulin therapy can be beneficial for patients with disabling autoimmune forms of dysautonomia, including small fiber polyneuropathy. IVIG showed improvement in approximately 80% of people with small fiber polyneuropathy. Caution is warranted, however, as patients with autonomic dysfunction develop aseptic meningitis or severe lingering headache when used in traditional fashion (1-2 g/kg given over 2-5 days).

Small fiber polyneuropathy may be co-morbid with:

  • Postural tachycardia syndrome
  • Orthostatic intolerance
  • Orthostatic hypotension
  • Inappropriate sinus tachycardia
  • Gastrointestinal dysmotility
  • Complex regional pain syndrome
  • Neurogenic bladder

In addition, dysautonomia may occur in association with

  • Primary antineuronal autoimmunity: antibodies to adrenergic and muscarinic receptors, ganglionic acetylcholine receptor, NMDA receptor and others
  • Systemic autoimmune disease and autonomic neuropathy including Sjogren syndrome and antiphospholipid syndrome
  • Many other autoimmune diseases, including:
    • Celiac disease
    • Hashimoto thyroiditis
    • Inflammatory bowel disease
    • Lupus
    • Multiple sclerosis
    • Myasthenia gravis
    • Rheumatoid arthritis
    • Spondylarthropathy
    • Systemic sclerosis

Sample IVIg Protocol for Suspected Autoimmune Related Dysautonomia

Taken from Schofield and Chemali. 2018. "How we treat autoimmune small fiber polyneuropathy with immunoglobulin therapy." European Neurology 80: 304-310.

ivig protocol

Dr. Schofield gave an excellent presentation on IVIG Therapy in Refractory Dysautonomia in 2017 with more detail than presented here.