Postural orthostatic tachycardia syndrome (POTS): State of the science and clinical care from a 2019 National Institutes of Health Expert Consensus Meeting - Part 1

Vernino, Steven; Bourne, Kate; Stiles, Lauren E.; Grubb, Blair P.; Fedorowski, Artur; Stewart, Julian M.; Arnold, Amy C.; Pace, Laura A.; Axelsson, Jonas; Boris, Jeffrey R.; Moak, Jeffrey P.; Goodman, Brent P.; Chémali, Kamal R.; Chung, Tae Hwan; Goldstein, David S.; Diedrich, André; Miglis, Mitchell G.; Cortez, Melissa; Miller, Amanda J.; Freeman, Roy; Biaggioni, Italo; Rowe, Peter C.; Sheldon, Robert S.; Shibao, Cyndya A.; Systrom, David M.; Cook, Glen; Doherty, Taylor; Abdallah, Hasan I.; Darbari, Anil; Raj, Satish R.

doi:10.1016/j.autneu.2021.102828

Cited by 167 publications

(199 citation statements)

References 115 publications

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“…Akin to heat stroke, several new hypotheses of ME/CFS suggest that vasoconstriction and hypoxia are central to its pathology—respectively focused on the consequence of ß2 adrenergic and M3 acetylcholine receptor autoantibodies ( 41 , 42 ), a potential reduction of ACE2 expression ( 43 ), and an abnormal immune response with a concomitant impact on endothelial function ( 44 ). In addition, postural orthostatic tachycardia syndrome (POTS) is a common comorbidity of ME/CFS ( 45 ), which is a complex disorder of the vascular system also associated with gastrointestinal dysfunctions ( 46 , 47 ). Impaired blood pressure variability and arrythmia have been observed in the patients with ME/CFS ( 48 , 49 ).…”

Section: Pathophysiological Mechanismsmentioning

confidence: 99%

Lessons From Heat Stroke for Understanding Myalgic Encephalomyelitis/Chronic Fatigue Syndrome

et al. 2021

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We here provide an overview of the pathophysiological mechanisms during heat stroke and describe similar mechanisms found in myalgic encephalomyelitis/chronic fatigue syndrome (ME/CFS). Both conditions are characterized by disturbed homeostasis in which inflammatory pathways play a central role. Splanchnic vasoconstriction, increased gut permeability, gut-related endotoxemia, systemic inflammatory response, central nervous system dysfunction, blood coagulation disorder, endothelial-cell injury, and mitochondrial dysfunction underlie heat stroke. These mechanisms have also been documented in ME/CFS. Moreover, initial transcriptomic studies suggest that similar gene expressions are altered in both heat stroke and ME/CFS. Finally, some predisposing factors for heat stroke, such as pre-existing inflammation or infection, overlap with those for ME/CFS. Notwithstanding important differences - and despite heat stroke being an acute condition - the overlaps between heat stroke and ME/CFS suggest common pathways in the physiological responses to very different forms of stressors, which are manifested in different clinical outcomes. The human studies and animal models of heat stroke provide an explanation for the self-perpetuation of homeostatic imbalance centered around intestinal wall injury, which could also inform the understanding of ME/CFS. Moreover, the studies of novel therapeutics for heat stroke might provide new avenues for the treatment of ME/CFS. Future research should be conducted to investigate the similarities between heat stroke and ME/CFS to help identify the potential treatments for ME/CFS.

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Section: Pathophysiological Mechanismsmentioning

confidence: 99%

Lessons From Heat Stroke for Understanding Myalgic Encephalomyelitis/Chronic Fatigue Syndrome

et al. 2021

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“…In addition, prognostication in POTS remains difficult due to the lack of specific outcome measures for this condition, and the lack of common data elements shared by investigators. Since POTS is clearly heterogeneous, advancements will require careful investigations of several different, but interacting, mechanisms which are described in the accompanying manuscript ( Vernino et al, 2021 ).…”

Section: Pathophysiology Of Potsmentioning

confidence: 99%

Postural orthostatic tachycardia syndrome (POTS): Priorities for POTS care and research from a 2019 National Institutes of Health Expert Consensus Meeting – Part 2

Raj

Bourne

Stiles

et al. 2021

Autonomic Neuroscience

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“…Postural orthostatic tachycardia syndrome (POTS) is a condition of orthostatic intolerance with a racing heart and a multitude of symptoms, induced upon standing and relieved when becoming supine [ 1 ]. Common nonspecific symptoms reported with POTS include exercise intolerance, fatigue, lightheadedness, palpitations, nausea, headache, diminished concentration (“brain fog”), near syncope, and syncope.…”

Section: Introductionmentioning

confidence: 99%

“…It is a debilitating disorder, affecting an estimated 1% of the population and likely more with the numerous reports in the literature of COVID-19 “long haulers” exhibiting many of the symptoms described by POTS patients; a number of these patients have actually been diagnosed with POTS [ 2 , 3 , 4 , 5 , 6 ]. The disorder was likely first described in 1871 by Da Costa, who described all of the features of POTS mentioned above; it was formally monikered in 1993 by Schondorf and Low, yet, even today, it is not well recognized in the medical community, nor has an etiology been established [ 1 , 7 ]. Unfortunately, as many as 75% of affected individuals are misdiagnosed or referred for psychological consultation [ 8 , 9 , 10 , 11 ].…”

Section: Introductionmentioning

confidence: 99%

“…Unfortunately, as many as 75% of affected individuals are misdiagnosed or referred for psychological consultation [ 8 , 9 , 10 , 11 ]. The diagnosis requires the presence of chronic orthostatic intolerance associated with an increased heart rate of ≥30 beats per minute (BPM) from the supine or sitting basal rate, or a rate that exceeds 120 BPM when standing or by an upright tilt test that occurs within 10 min [ 1 , 12 ]. There is also an absence of orthostatic hypotension, a duration of symptoms for at least 6 months, and the absence of a number of conditions that could explain sinus tachycardia [ 1 ].…”

Section: Introductionmentioning

confidence: 99%

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Platelet Storage Pool Deficiency and Elevated Inflammatory Biomarkers Are Prevalent in Postural Orthostatic Tachycardia Syndrome

Gunning

Kramer

Cichocki

et al. 2022

Cells

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A significant number of postural orthostatic tachycardia syndrome (POTS) patients have platelet delta granule storage pool deficiency (δ-SPD). The etiology of POTS is unknown but a number of laboratories, including ours, have reported elevations of G-protein-coupled adrenergic receptor and muscarinic acetylcholine receptor autoantibodies in POTS patients, detected by a variety of techniques, suggesting that the disorder is an autoimmune condition. Thus, it could also be considered an inflammatory disease. In a pilot study, we investigated a limited number of platelet-related cytokines and chemokines and discovered many that were elevated. This case–control study validates our pilot study results that POTS patients have an activated innate immune system. Plasma of 35 POTS patients and 35 patients with unexplained bleeding symptoms and categorized as “non-POTS” subjects was analyzed by multiplex flow cytometry to quantify 16 different innate immune system cytokines and chemokines. Electron microscopy was used to quantify platelet dense granules. Ten of 16 biomarkers of inflammation were elevated in plasma from POTS patients compared to non-POTS subjects, with most of the differences extremely significant, with p values < 0.0001. Of particular interest were elevations of IL-1β and IL-18 and decreased or normal levels of type 1 interferons in POTS patients, suggesting that the etiology of POTS might be autoinflammatory. All POTS patients had δ-SPD. With a growing body of evidence that POTS is an autoimmune disease and having elevations of the innate immune system, our results suggest a potential T-cell-mediated autoimmunity in POTS characteristic of a mixed-pattern inflammatory disease similar to rheumatoid arthritis.

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Postural orthostatic tachycardia syndrome (POTS): State of the science and clinical care from a 2019 National Institutes of Health Expert Consensus Meeting - Part 1

Cited by 167 publications

References 115 publications

Lessons From Heat Stroke for Understanding Myalgic Encephalomyelitis/Chronic Fatigue Syndrome

Lessons From Heat Stroke for Understanding Myalgic Encephalomyelitis/Chronic Fatigue Syndrome

Postural orthostatic tachycardia syndrome (POTS): Priorities for POTS care and research from a 2019 National Institutes of Health Expert Consensus Meeting – Part 2

Platelet Storage Pool Deficiency and Elevated Inflammatory Biomarkers Are Prevalent in Postural Orthostatic Tachycardia Syndrome

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