Maria Roma1, Colleen L Marden2, Inge De Wandele3, Clair A Francomano4, Peter C Rowe5. 1. Department of Pediatrics, Johns Hopkins University School of Medicine, 200 N. Wolfe St., Room 2077, Baltimore, MD 21287, USA. 2. Department of Pediatrics, Johns Hopkins University School of Medicine, 200 N. Wolfe St., Room 2077, Baltimore, MD 21287, USA. Electronic address: cmarden1@jhmi.edu. 3. Department of Rehabilitation Sciences and Physiotherapy, Ghent University, Belgium. Electronic address: inge.dewandele@ugent.be. 4. Harvey Institute for Human Genetics, 6701 North Charles St., Suite 2326, Towson, MD 21204, USA. Electronic address: CFrancomano@gbmc.org. 5. Department of Pediatrics, Johns Hopkins University School of Medicine, 200 N. Wolfe St., Room 2077, Baltimore, MD 21287, USA. Electronic address: prowe@jhmi.edu.
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.
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.
Authors: Amanda J Miller; Lauren E Stiles; Timothy Sheehan; Rebecca Bascom; Howard P Levy; Clair A Francomano; Amy C Arnold Journal: Auton Neurosci Date: 2020-01-10 Impact factor: 3.145
Authors: Cortney Gensemer; Randall Burks; Steven Kautz; Daniel P Judge; Mark Lavallee; Russell A Norris Journal: Dev Dyn Date: 2020-08-17 Impact factor: 3.780
Authors: Steven Vernino; Kate M Bourne; Lauren E Stiles; Blair P Grubb; Artur Fedorowski; Julian M Stewart; Amy C Arnold; Laura A Pace; Jonas Axelsson; Jeffrey R Boris; Jeffrey P Moak; Brent P Goodman; Kamal R Chémali; Tae H Chung; David S Goldstein; Andre Diedrich; Mitchell G Miglis; Melissa M Cortez; Amanda J Miller; Roy Freeman; Italo Biaggioni; Peter C Rowe; Robert S Sheldon; Cyndya A Shibao; David M Systrom; Glen A Cook; Taylor A Doherty; Hasan I Abdallah; Anil Darbari; Satish R Raj Journal: Auton Neurosci Date: 2021-06-05 Impact factor: 2.355