Postural tachycardia syndrome (POTS) and antiphospholipid syndrome (APS): What do we know so far?

Rev Neurol (Paris). 2022 Apr;178(4):306-314. doi: 10.1016/j.neurol.2021.10.006. Epub 2021 Dec 9.

Abstract

As part of the non-criteria clinical manifestations, postural orthostatic tachycardia syndrome (POTS), a multisystem autonomic dysfunction, can co-exist with antiphospholipid syndrome (APS). Several pieces of evidence hint on the autoimmune basis of POTS, and its possible association with several autoimmune diseases, including APS. Indeed, the evidence exists in the etiologies, symptomatology, and treatment options. Although infections, viral ones in particular, stress, and pregnancy are etiologies to both POTS and APS, the exact pathophysiological connection is still to be studied taking into consideration the activity of cytokines in both diseases. Nevertheless, certain immunomodulatory treatments used for the catastrophic or obstetrical forms of APS, such as intravenous immunoglobulins (IVIG) and steroids, have been also used for the treatment of POTS resistant to classical treatments. Therefore, our review aims to highlight the association between POTS and APS, shedding light on the common etiologies explaining the pathophysiology of the two disorders, the diagnostic approach to POTS as a possible clinical criterion of APS, and the treatment of APS in the context of treating POTS.

Keywords: Anticoagulation; Antiphospholipid antibodies; Antiphospholipid syndrome; Autonomic dysfunction; Cytokines; Postural orthostatic tachycardia syndrome.

Publication types

  • Review

MeSH terms

  • Antiphospholipid Syndrome* / complications
  • Antiphospholipid Syndrome* / diagnosis
  • Antiphospholipid Syndrome* / therapy
  • Autoimmune Diseases* / complications
  • Female
  • Humans
  • Immunoglobulins, Intravenous / therapeutic use
  • Postural Orthostatic Tachycardia Syndrome* / diagnosis
  • Postural Orthostatic Tachycardia Syndrome* / epidemiology
  • Postural Orthostatic Tachycardia Syndrome* / etiology
  • Pregnancy
  • Primary Dysautonomias*

Substances

  • Immunoglobulins, Intravenous