Prevalence of hypermobile Ehlers-Danlos syndrome in postural orthostatic tachycardia syndrome
Open accessAbstract
Despite well-established clinical associations between Hypermobile Ehlers-Danlos syndrome (hEDS) and postural orthostatic tachycardia syndrome (POTS), the precise prevalence is unknown. We therefore evaluated for hEDS in 91 POTS participants using the 2017 hEDS diagnostic checklist, which has three major criteria: 1) generalized joint hypermobility (Beighton score), 2) systemic features, family history, and 3) absence of exclusion criteria. Overall, 28 out of 91 POTS participants (31%) met clinical criteria for hEDS. An additional 24% of participants had generalized joint hypermobility without meeting hEDS criteria. Identifying the prevalence of hEDS in POTS is important for understanding possible mechanisms connecting these two syndromes.
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The prevalence of hEDS appears to be higher in PoTS cohorts compared with the general population, with 31% of PoTS patients meeting the 2017 diagnostic criteria for hEDS.
“The prevalence of hEDS also appears to be higher in PoTS cohorts compared with the general population, with 31% of PoTS patients meeting the 2017 diagnositc criteria for hEDS. 72”
From Hypermobile Ehlers–Danlos syndrome and disorders of the gastrointestinal tract: What the gastroenterologist needs to know (2022) · cites this paper as [72]
Approximately 40% of POTS patients experience migraine, 20-30% meet criteria for hypermobile Ehlers-Danlos syndrome, and about 15% carry a diagnosis of co-morbid autoimmune disease.
“Current estimates derived from small samples or uncontrolled survey data suggest that approximately 40% of POTS patients experience migraine, 20–30% meet the diagnostic criteria for hypermobile Ehlers-Danlos syndrome (Miller et al., 2020; Roma et al., 2018), and about 15% carry a diagnosis of a co-morbid autoimmune disease.”
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 Miller et al., 2020
Orthostatic intolerance and tachycardia can be found in up to 40% of patients with hypermobility spectrum disorder or hypermobile Ehlers-Danlos syndrome.
“Conversely, orthostatic intolerance and tachycardia can be found in up to 40% of patients with hypermobility spectrum disorder or hypermobile Ehlers-Danlos Syndrome (Miller et al., 2020; Roma 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 Miller et al., 2020
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The 2017 international classification of the Ehlers–Danlos syndromes
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Self-citationOrthostatic intolerance and chronic fatigue syndrome associated with Ehlers-Danlos syndrome
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Ehlers–Danlos Syndrome and Postural Tachycardia Syndrome: A relationship study
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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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- Authors
- Miller, A. J., Stiles, L. E., Sheehan, T., Bascom, R., Levy, H. P., Francomano, C. A., & Arnold, A. C.
- Affiliations
- Pennsylvania State University (US)
- Stony Brook School (US)
- Medical University of South Carolina (US)
- Johns Hopkins University (US)
- Indiana University Indianapolis (US)
- Corresponding author
- Miller, Amanda J.
- ORCID
- Venue
- Autonomic Neuroscience
- Publisher
- Elsevier BV
- Published
- 2020-03
- Volume
- 224
- Pages
- 102637
- ISSN
- 1566-0702 (ISSN-L)
- PMC
- PMC7282488
- OpenAlex
- W2999361324
- Cited by
- 102
- References
- 7
- Citations by year
- 2026: 19 · 2025: 19 · 2024: 10 · 2023: 10 · 2022: 9 · 2021: 32 · 2020: 2
- FWCI
- 12.11
- Type
- journal article
- Language
- en
- Open access
- green
- www.ncbi.nlm.nih.gov
- Locations
- Autonomic Neuroscience (publishedVersion)
- PubMed (publishedVersion)
- PubMed Central (acceptedVersion)
- ScholarSphere (Penn State Libraries) (submittedVersion, cc-by-nc-nd)
- IUScholarWorks (Indiana University) (submittedVersion)
- Funders
- National Institutes of Health
- American Heart Association
- Topics
- Health Sciences › Medicine › Surgery › Cardiovascular Syncope and Autonomic Disorders
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Genetics › Connective tissue disorders research
- Health Sciences › Medicine › Psychiatry and Mental health › Fibromyalgia and Chronic Fatigue Syndrome Research
- Keywords
- hypermobile Ehlers-Danlos syndrome, postural orthostatic tachycardia syndrome, prevalence, generalized joint hypermobility, Beighton score, comorbidities, autonomic dysfunction
- MeSH terms
- Adolescent, Adult, Ehlers-Danlos Syndrome, Female, Humans, Joint Instability, Male, Middle Aged, Prevalence, Postural Orthostatic Tachycardia Syndrome, Young Adult
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