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Immunotherapy with subcutaneous immunoglobulin or plasmapheresis in patients with postural orthostatic tachycardia syndrome (POTS)

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Abstract

Background

Postural orthostatic tachycardia syndrome (POTS), one of the most common autonomic disorders, is associated with significant morbidity and functional impairment. Although several possible etiologies have been proposed, autoimmunity has emerged as one of the leading causes with various specific and non-specific antibodies identified in patients with POTS. Treatment with intravenous immunoglobulin has been previously described. We present a case series of patients with severe POTS refractory to the standard pharmacologic and non-pharmacologic therapies treated with immunotherapy consisting of either subcutaneous immunoglobulin (SCIG) therapy or plasmapheresis (PLEX) and report their treatment outcomes.

Methods

Clinical history of 7 patients with POTS who were treated with SCIG or PLEX was reviewed. Response to treatment was assessed using COMPASS 31 and functional ability scale (FAS), completed by patients retrospectively, pre- and 3–12 months post-treatment with SCIG or PLEX.

Results

All patients improved following SCIG or PLEX with an average 50% reduction in COMPASS 31 score and 217% increase in FAS scores. Six out of seven patients were able to reduce or discontinue oral medications for POTS, and five patients were able to return to work or school. Four patients had skin biopsy or quantitative sudomotor axon reflex test (QSART)-proven small fiber neuropathy, and five had various positive antibodies at low titers.

Conclusion

Patients with severe, treatment-refractory POTS experienced significant functional improvement with reduction in the autonomic symptoms following immunotherapy with SCIG or PLEX. Randomized controlled trials of SCIG and/or PLEX are needed to determine the efficacy and safety of these long-term therapies in patients with POTS.

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Data availability statement

Svetlana Blitshteyn takes full responsibility for the data, the analyses and interpretation, and the conduct of the research, has full access to all of the data, and has the right to publish any and all data separate and apart from any sponsor. The data that support the findings of this study are available from Svetlana Blitshteyn upon reasonable request.

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Funding

None.

Svetlana Blitshteyn takes full responsibility for the data, the analyses and interpretation, and the conduct of the research, has full access to all of the data, and has the right to publish any and all data separate and apart from any sponsor. The data that support the findings of this study are available from Svetlana Blitshteyn upon reasonable request.

Author information

Authors and Affiliations

  1. Department of Psychiatry, University of Central Florida/HCA GME Consortium, Orlando, FL, USA

    Katrina Kesterson

  2. Department of Medicine, University of Colorado School of Medicine, Aurora, CO, USA

    Jill Schofield

  3. Department of Neurology, University at Buffalo Jacobs School of Medicine and Biomedical Sciences, Buffalo, NY, USA

    Svetlana Blitshteyn

Authors
  1. Katrina Kesterson
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  2. Jill Schofield
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  3. Svetlana Blitshteyn
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Contributions

KK—conceptualization, data curation, formal analysis, and writing. JS—conceptualization (supporting), formal analysis, and writing—review and editing. SB—conceptualization (lead); formal analysis, writing—review and editing, and supervision.

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Correspondence to Svetlana Blitshteyn.

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Conflicts of interest

Katrina Kesterson—reports no disclosures or conflicts of interest. Jill Schofield—reports no disclosures or conflicts of interest. Svetlana Blitshteyn—reports no conflicts of interest. Svetlana Blitshteyn has served as a medical expert witness on cases of POTS in the U.S. Court of Federal Claims and for the U.S. Department of Labor.

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Kesterson, K., Schofield, J. & Blitshteyn, S. Immunotherapy with subcutaneous immunoglobulin or plasmapheresis in patients with postural orthostatic tachycardia syndrome (POTS). J Neurol 270, 233–239 (2023). https://doi.org/10.1007/s00415-022-11344-z

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  • DOI: https://doi.org/10.1007/s00415-022-11344-z

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