Abstract
Objective: To review the association between orthostatic intolerance syndromes and both joint hypermobility and Ehlers-Danlos syndrome, and to propose reasons for identifying hereditary connective tissue disorders in those with orthostatic intolerance in the context of both clinical care and research. Methods: We searched the published peer-reviewed medical literature for papers reporting an association between joint hypermobility or Ehlers-Danlos syndrome and orthostatic intolerance. Results: We identified 10 relevant papers. Although methodological variability between studies introduces some limitations, the published literature consistently identifies a significantly higher prevalence of orthostatic intolerance symptoms in patients with joint hypermobility or Ehlers-Danlos syndrome than in healthy controls, and a significantly higher prevalence of cardiovascular and autonomic abnormalities both at rest and during orthostatic challenge. Postural tachycardia syndrome is the most commonly recognized circulatory disorder. The severity of orthostatic symptoms in those with EDS correlates with impairments in quality of life. Conclusion: There is a strong association between several forms of cardiovascular dysfunction, most notably postural tachycardia syndrome, and joint hypermobility or Ehlers-Danlos syndrome. We propose that recognition of joint hypermobility and Ehlers-Danlos syndrome among those with orthostatic intolerance syndromes has the potential to improve clinical care and the validity of research findings.
| Original language | English (US) |
|---|---|
| Journal | Autonomic Neuroscience: Basic and Clinical |
| DOIs | |
| State | Accepted/In press - Jan 1 2018 |
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Keywords
- Chronic fatigue syndrome
- Ehlers-Danlos syndrome
- Hypotension
- Joint hypermobility
- Postural tachycardia syndrome
- Syncope, neurally mediated, vasovagal
ASJC Scopus subject areas
- Endocrine and Autonomic Systems
- Clinical Neurology
- Cellular and Molecular Neuroscience
Cite this
Postural tachycardia syndrome and other forms of orthostatic intolerance in Ehlers-Danlos syndrome. / Roma, Maria; Marden, Colleen L.; De Wandele, Inge; Francomano, Clair Ann; Rowe, Peter.
In: Autonomic Neuroscience: Basic and Clinical, 01.01.2018.Research output: Contribution to journal › Article
}
TY - JOUR
T1 - Postural tachycardia syndrome and other forms of orthostatic intolerance in Ehlers-Danlos syndrome
AU - Roma, Maria
AU - Marden, Colleen L.
AU - De Wandele, Inge
AU - Francomano, Clair Ann
AU - Rowe, Peter
PY - 2018/1/1
Y1 - 2018/1/1
N2 - Objective: To review the association between orthostatic intolerance syndromes and both joint hypermobility and Ehlers-Danlos syndrome, and to propose reasons for identifying hereditary connective tissue disorders in those with orthostatic intolerance in the context of both clinical care and research. Methods: We searched the published peer-reviewed medical literature for papers reporting an association between joint hypermobility or Ehlers-Danlos syndrome and orthostatic intolerance. Results: We identified 10 relevant papers. Although methodological variability between studies introduces some limitations, the published literature consistently identifies a significantly higher prevalence of orthostatic intolerance symptoms in patients with joint hypermobility or Ehlers-Danlos syndrome than in healthy controls, and a significantly higher prevalence of cardiovascular and autonomic abnormalities both at rest and during orthostatic challenge. Postural tachycardia syndrome is the most commonly recognized circulatory disorder. The severity of orthostatic symptoms in those with EDS correlates with impairments in quality of life. Conclusion: There is a strong association between several forms of cardiovascular dysfunction, most notably postural tachycardia syndrome, and joint hypermobility or Ehlers-Danlos syndrome. We propose that recognition of joint hypermobility and Ehlers-Danlos syndrome among those with orthostatic intolerance syndromes has the potential to improve clinical care and the validity of research findings.
AB - Objective: To review the association between orthostatic intolerance syndromes and both joint hypermobility and Ehlers-Danlos syndrome, and to propose reasons for identifying hereditary connective tissue disorders in those with orthostatic intolerance in the context of both clinical care and research. Methods: We searched the published peer-reviewed medical literature for papers reporting an association between joint hypermobility or Ehlers-Danlos syndrome and orthostatic intolerance. Results: We identified 10 relevant papers. Although methodological variability between studies introduces some limitations, the published literature consistently identifies a significantly higher prevalence of orthostatic intolerance symptoms in patients with joint hypermobility or Ehlers-Danlos syndrome than in healthy controls, and a significantly higher prevalence of cardiovascular and autonomic abnormalities both at rest and during orthostatic challenge. Postural tachycardia syndrome is the most commonly recognized circulatory disorder. The severity of orthostatic symptoms in those with EDS correlates with impairments in quality of life. Conclusion: There is a strong association between several forms of cardiovascular dysfunction, most notably postural tachycardia syndrome, and joint hypermobility or Ehlers-Danlos syndrome. We propose that recognition of joint hypermobility and Ehlers-Danlos syndrome among those with orthostatic intolerance syndromes has the potential to improve clinical care and the validity of research findings.
KW - Chronic fatigue syndrome
KW - Ehlers-Danlos syndrome
KW - Hypotension
KW - Joint hypermobility
KW - Postural tachycardia syndrome
KW - Syncope, neurally mediated, vasovagal
UR - http://www.scopus.com/inward/record.url?scp=85042865725&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=85042865725&partnerID=8YFLogxK
U2 - 10.1016/j.autneu.2018.02.006
DO - 10.1016/j.autneu.2018.02.006
M3 - Article
JO - Autonomic Neuroscience: Basic and Clinical
JF - Autonomic Neuroscience: Basic and Clinical
SN - 1566-0702
ER -