Orthostatic intolerance and chronic fatigue syndrome associated with Ehlers-Danlos syndrome
Open accessAbstract
OBJECTIVE: To report chronic fatigue syndrome (CFS) associated with both Ehlers-Danlos syndrome (EDS) and orthostatic intolerance. STUDY DESIGN: Case series of adolescents referred to a tertiary clinic for the evaluation of CFS. All subjects had 2-dimensional echocardiography, tests of orthostatic tolerance, and examinations by both a geneticist and an ophthalmologist. RESULTS: Twelve patients (11 female), median age 15.5 years, met diagnostic criteria for CFS and EDS, and all had either postural tachycardia or neurally mediated hypotension in response to orthostatic stress. Six had classical-type EDS and 6 had hypermobile-type EDS. CONCLUSIONS: Among patients with CFS and orthostatic intolerance, a subset also has EDS. We propose that the occurrence of these syndromes together can be attributed to the abnormal connective tissue in dependent blood vessels of those with EDS, which permits veins to distend excessively in response to ordinary hydrostatic pressures. This in turn leads to increased venous pooling and its hemodynamic and symptomatic consequences. These observations suggest that a careful search for hypermobility and connective tissue abnormalities should be part of the evaluation of patients with CFS and orthostatic intolerance syndromes.
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Other studies suggest joint hypermobility probably precedes and predisposes to both temporomandibular disorders and POTS.
“Other studies, however, suggest that joint hypermobility likely precedes and predisposes to both TMD (De Coster et al., 2005; Kavuncu et al., 2005; Deodato et al., 2006; Pasinato et al., 2011) and POTS (Rowe et al., 1999).”
From Postural tachycardia syndrome and other forms of orthostatic intolerance in Ehlers-Danlos syndrome (2018) · cites this paper as Rowe et al., 1999
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Self-citation
- Authors
- Rowe, P. C., Barron, D. F., Calkins, H., Maumenee, I. H., Tong, P. Y., & Geraghty, M. T.
- Affiliations
- Johns Hopkins University (US)
- Venue
- The Journal of Pediatrics
- Publisher
- Elsevier BV
- Published
- 1999-10
- Volume
- 135
- Issue
- 4
- Pages
- 494-499
- ISSN
- 0022-3476 (ISSN-L)
- OpenAlex
- W2033921066
- Cited by
- 230
- References
- 49
- Citations by year
- 2026: 14 · 2025: 17 · 2024: 17 · 2023: 8 · 2022: 17 · 2021: 10 · 2020: 8 · 2019: 6 · 2018: 12 · 2017: 15 · 2016: 8 · 2015: 5 · 2014: 11 · 2013: 12 · 2012: 6
- FWCI
- 2.81
- Type
- journal article
- Language
- en
- Open access
- hybrid
- doi.org
- Licence
- Locations
- The Journal of Pediatrics (publishedVersion, cc-by-nc-nd)
- PubMed (publishedVersion)
- Funders
- National Institutes of Health (5r01ai039500-03)
- National Institute of Allergy and Infectious Diseases (AI 39500)
- Topics
- Health Sciences › Medicine › Surgery › Cardiovascular Syncope and Autonomic Disorders
- Health Sciences › Medicine › Psychiatry and Mental health › Fibromyalgia and Chronic Fatigue Syndrome Research
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Genetics › Connective tissue disorders research
- Keywords
- Ehlers-Danlos syndrome, chronic fatigue syndrome, orthostatic intolerance, postural orthostatic tachycardia syndrome, hypermobile Ehlers-Danlos syndrome, connective tissue abnormalities, venous pooling, adolescents
- MeSH terms
- Adolescent, Adult, Blood Pressure, Child, Ehlers-Danlos Syndrome, Female, Heart Rate, Humans, Hypotension, Orthostatic, Male, Fatigue Syndrome, Chronic
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