Intravenous Immunoglobulin Therapy in Refractory Autoimmune Dysautonomias: A Retrospective Analysis of 38 Patients
Abstract
Background Intravenous immunoglobulin (IVIG) has recognized efficacy in autoimmune peripheral nerve disorders, but there has been limited study of the use of IVIG in autoimmune dysautonomias. Study question To determine the efficacy and safety of IVIG in patients with disabling, refractory autoimmune dysautonomias, including patients with postural tachycardia syndrome and gastrointestinal dysmotility. Study design Patients with one or more autonomic disorder(s) and persistent serological evidence for autoimmunity who were unable to work or attend school despite usual treatments for dysautonomia were treated with IVIG for at least 3 months at a dose of at least 1 gm/kg monthly. Measures and outcomes Outcome measures included the composite autonomic symptom scale 31 survey and a functional ability score. Results There were 38 patients, 84% female and mean age of 28.4 years. Of patients, 83.5% improved on IVIG as defined by at least 20% improvement in the composite autonomic symptom scale 31 and/or functional ability score. The mean pretreatment functional ability score was 21% (mostly bedridden), which improved to a mean of 74% (nearing able to return to work/school) for responsive patients after at least 1 year of IVIG. The mean time to the first sign of response was 5.3 weeks. There were no serious adverse events. The Mayo autoimmune dysautonomia panel antibodies and traditional Sjögren antibodies were present in only 13% and 8% of patients, respectively, but antiphospholipid…
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Anecdotal evidence from case reports and retrospective case series suggests that IVIG may be effective in refractory POTS and other suspected autoimmune dysautonomia.
“Further, a number of case reports, and retrospective case series have provided anecdotal evidence suggesting efficacy of IVIG in the treatment of refractory POTS and other forms of suspected autoimmune dysautonomia (Goodman, 2014; Schofield and Chemali, 2019; Weinstock et al., 2018).”
From Postural orthostatic tachycardia syndrome (POTS): State of the science and clinical care from a 2019 National Institutes of Health Expert Consensus Meeting - Part 1 (2021) · cites this paper as Schofield and Chemali, 2019
In a series of 38 POTS patients with gastrointestinal dysmotility, antiphospholipid antibodies were found in 76% and novel Sjögren antibodies in 42%.
“In a series of 38 patients with POTS and gastrointestinal dysmotility, antiphospholipid antibodies were found in 76%, whereas novel Sjogren antibodies in 42% of all subjects [41].”
From Postural orthostatic tachycardia syndrome: clinical presentation, aetiology and management (2019) · cites this paper as [41]
Other autoantibodies with a proposed aetiological role in POTS include antibodies against nicotinic acetylcholine receptors in autonomic ganglia and Sjögren autoantibodies.
“Other autoantibodies with a proposed aetiological role in POTS include antibodies against nicotinic acetylcholine-receptor in autonomic ganglia [26] and Sj€ ogren autoantibodies [41,49], detected in POTS through antibody screening programmes.”
From Postural orthostatic tachycardia syndrome: clinical presentation, aetiology and management (2019) · cites this paper as [41]
In patients with POTS, thyroid autoantibodies-against the TSH receptor, thyroglobulin, and thyroid peroxidase-occur at a higher prevalence than in the general population.
“Thyroid gland [40, 41]”
From Postural orthostatic tachycardia syndrome: clinical presentation, aetiology and management (2019) · cites this paper as [41]
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Dysautonomia in the joint hypermobility syndrome
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Postural orthostatic tachycardia syndrome: clinical presentation, aetiology and management
Fedorowski, A. (2019) · Journal of Internal Medicine · doi:10.1111/joim.12852 · Cited by 352 · see citing works
Vernino, S., Bourne, K. M., Stiles, L. E., Grubb, B. P., Fedorowski, A., Stewart, J. M., Arnold, A. C., Pace, L. A., Axelsson, J., Boris, J. R., et al. (2021) · Autonomic Neuroscience · doi:10.1016/j.autneu.2021.102828 · Cited by 304 · see citing works
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Sinus Tachycardia: a Multidisciplinary Expert Focused Review
Mayuga, K. A., Fedorowski, A., Ricci, F., Gopinathannair, R., Dukes, J. W., Gibbons, C., Hanna, P., Sorajja, D., Chung, M., Benditt, D., et al. (2022) · Circulation: Arrhythmia and Electrophysiology · doi:10.1161/circep.121.007960 · Cited by 59 · see citing works
- Authors
- Schofield, J. R., & Chemali, K. R.
- Affiliations
- University of Colorado Anschutz (US)
- Department of Neurology, Eastern Virginia Medical School, Norfolk, VA.
- Corresponding author
- Schofield, Jill R.
- ORCID
- Schofield, Jill R.
- Venue
- American Journal of Therapeutics
- Published
- 2019-09
- Volume
- 26
- Issue
- 5
- Pages
- e570-e582
- ISSN
- 1075-2765 (ISSN-L)
- OpenAlex
- W2804669447
- Cited by
- 55
- References
- 2
- Citations by year
- 2026: 3 · 2025: 3 · 2024: 11 · 2023: 5 · 2022: 8 · 2021: 13 · 2020: 8 · 2019: 2 · 2018: 2
- FWCI
- 6.54
- Type
- journal article
- Language
- en
- Open access
- closed
- Locations
- American Journal of Therapeutics (publishedVersion)
- PubMed (publishedVersion)
- Topics
- Health Sciences › Medicine › Surgery › Cardiovascular Syncope and Autonomic Disorders
- Health Sciences › Medicine › Neurology › Peripheral Neuropathies and Disorders
- Health Sciences › Medicine › Neurology › Autoimmune Neurological Disorders and Treatments
- Keywords
- intravenous immunoglobulin, postural orthostatic tachycardia syndrome, gastrointestinal motility disorders, antiphospholipid antibodies, functional capacity, treatment response, adverse events
- MeSH terms
- Adolescent, Adult, Child, Dose-Response Relationship, Drug, Female, Humans, Immunologic Factors, Male, Middle Aged, Retrospective Studies, Immunoglobulins, Intravenous, Treatment Outcome, Antibodies, Antiphospholipid, Autoimmune Diseases of the Nervous System, Primary Dysautonomias, Young Adult
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