Postural Orthostatic Tachycardia Syndrome (POTS) – A novel member of the autoimmune family

Background Patients with postural tachycardia syndrome (POTS) often have gastrointestinal (GI) symptoms. Occasionally, these symptoms can be so severe that nonoral nutrition/hydration support (NONHS), including intravenous fluids (IVFs), enteral nutrition (EN), and parenteral nutrition (PN), becomes necessary. Methods This is a retrospective cohort study of adult patients diagnosed with POTS at the Mayo Clinic Arizona from January 2010 to January 2017 with a minimum of 6 months of follow up. Demographic information, symptomatology, medications, GI testing, autonomic and autoantibody testing, and healthcare utilization data were abstracted from the electronic medical record. Results Three‐hundred thirty‐two patients with POTS were included, of which 32 required NONHS. Patients receiving NONHS were more likely to be female; have lower body mass index; have GI symptoms including nausea, vomiting, diarrhea, and constipation; have abdominal pain; use opiates; have delayed gastric emptying; see more specialists; and be seen in an emergency room or be hospitalized for symptoms. Of these patients, 21 (66%) required IVF, 19 (59%) required EN, and 9 (28%) required PN. Six (19%) patients required all 3 NONHS modalities at some point during their follow‐up period. Conclusions NONHS may be required in a subset of patients with POTS. Those receiving NONHS have more severe symptoms and abnormal GI motility and autonomic testing and exhibit greater healthcare utilization. Management of these patients is complex and challenging and requires a multidisciplinary approach. Further prospective studies are needed to identify optimal management strategies.

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“…Autoimmunity has been implicated in the pathogenesis of POTS . We found no major differences in autoantibody testing.…”

Section: Discussioncontrasting

confidence: 42%

Factors Associated With Use of Nonoral Nutrition and Hydration Support in Adult Patients With Postural Tachycardia Syndrome

Tseng

Traub

Harris

et al. 2018

J Parenter Enteral Nutr

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“…A recent study showed that serum levels of autoantibodies to several autonomic receptors were higher in ME/CFS patients than in healthy controls (41). Similar autoantibodies were detected in postural orthostatic tachycardia syndrome (42) and in complex regional pain syndrome (43), conditions with some resemblances to ME/CFS, affecting fertile women after a triggering event (44,45). Likewise, autoantibodies in ME/CFS may interfere with key metabolic signaling pathways involving factors such as HIF1 (24) and PPARs, as well as AMPK, which was abnormally activated in skeletal muscle cells of ME/CFS patients (46).…”

Section: Discussionmentioning

confidence: 81%

Metabolic profiling indicates impaired pyruvate dehydrogenase function in myalgic encephalopathy/chronic fatigue syndrome

et al. 2016

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“…Moreover, Blitshteyn recently determined the high prevalence of positive ANA and other autoantibodies and the high prevalence of comorbid autoimmune disorders that exist in patients with POTS 8, 9. Sandroni and Low demonstrated occasional positivity to gAChR antibodies in POTS patients 10.…”

Section: Introductionmentioning

confidence: 99%

Autoimmune postural orthostatic tachycardia syndrome

Watari

Nakane

Mukaino

et al. 2018

Ann Clin Transl Neurol

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The aim of this study was to evaluate the association between postural orthostatic tachycardia syndrome (POTS) and circulating antiganglionic acetylcholine receptor (gAChR) antibodies. We reviewed clinical assessments of Japanese patients with POTS, and determined the presence of gAChR antibodies in serum samples from those patients. Luciferase immunoprecipitation systems detected anti‐gAChR α3 and β4 antibodies in the sera from POTS (29%). Antecedent infections were frequently reported in patients in POTS patients. Moreover, autoimmune markers and comorbid autoimmune diseases were also frequent in seropositive POTS patients. Anti‐gAChR antibodies were detectable in significant number of patients with POTS, and POTS entailed the element of autoimmune basis.

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Postural Orthostatic Tachycardia Syndrome (POTS) – A novel member of the autoimmune family

Cited by 48 publications

References 40 publications

Factors Associated With Use of Nonoral Nutrition and Hydration Support in Adult Patients With Postural Tachycardia Syndrome

Factors Associated With Use of Nonoral Nutrition and Hydration Support in Adult Patients With Postural Tachycardia Syndrome

Metabolic profiling indicates impaired pyruvate dehydrogenase function in myalgic encephalopathy/chronic fatigue syndrome

Autoimmune postural orthostatic tachycardia syndrome

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