Dysautonomia in the joint hypermobility syndrome
Abstract
Purpose Extraarticular manifestations of the joint hypermobility syndrome may include the peripheral nervous system. The purpose of this study was to investigate autonomic function in patients with this syndrome.Methods Forty-eight patients with the joint hypermobility syndrome who fulfilled the 1998 Brighton criteria and 30 healthy control subjects answered a clinical questionnaire designed to evaluate the frequency of complaints related to the autonomic nervous system. Next, 27 patients and 21 controls underwent autonomic evaluation: orthostatic testing, cardiovascular vagal and sympathetic functions, catecholamine levels, and adrenoreceptor responsiveness.Results Symptoms related to the autonomic nervous system, such as syncope and presyncope, palpitations, chest discomfort, fatigue, and heat intolerance, were significantly more common among patients. Orthostatic hypotension, postural orthostatic tachycardia syndrome, and uncategorized orthostatic intolerance were found in 78% (21/27) of patients compared with in 10% (2/21) of controls. Patients with the syndrome had a greater mean (+/- SD) drop in systolic blood pressure during hyperventilation than did controls (-11 +/- 7 mm Hg vs. -5 +/- 5 mm Hg, P = 0.02) and a greater increase in systolic blood pressure after a cold pressor test (19 +/- 10 mm Hg vs. 11 +/- 13 mm Hg, P = 0.06). Patients with the syndrome also had evidence of alpha-adrenergic (as assessed by administration of phenylephrine) and beta-adrenergic hyperresponsiveness (as assessed by administration of isoproterenol).Conclusion The autonomic nervous system-related symptoms of the patients have a pathophysiological basis, which suggests that dysautonomia is an extraarticular manifestation in the joint hypermobility syndrome.
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Hypermobility is associated with dysautonomia, typically PoTS, symptoms of which have phenomenological overlap with anxiety.
“Hypermobility is associated with dysautonomia,47 typically PoTS, symptoms of which have phenomenological overlap with anxiety.26”
From Variant connective tissue (joint hypermobility) and its relevance to depression and anxiety in adolescents: a cohort-based case–control study (2022) · cites this paper as [47]
HSD/hEDS patients are almost three times more likely than healthy controls to experience presyncopal symptoms (41% vs 15%).
“For example, they are almost three times more likely than healthy controls to experience presyncopal symptoms (41% vs 15%)”
From Hypermobile Ehlers–Danlos syndrome and disorders of the gastrointestinal tract: What the gastroenterologist needs to know (2022) · cites this paper as [63]
HSD/hEDS patients experience orthostatic intolerance frequently (94% of HSD/hEDS in one study).
“and experience orthostatic intolerance frequently (94% of HSD/hEDS in one study). 15 , 63 , 65”
From Hypermobile Ehlers–Danlos syndrome and disorders of the gastrointestinal tract: What the gastroenterologist needs to know (2022) · cites this paper as [63]
The prevalence of PoTS in HSD/hEDS cohorts ranges from 15% to 41%.
“The prevalence of PoTS in HSD/hEDS cohorts ranges from 15% to 41%. 36 , 63 , 64 , 70 , 71 , 72”
From Hypermobile Ehlers–Danlos syndrome and disorders of the gastrointestinal tract: What the gastroenterologist needs to know (2022) · cites this paper as [63]
Symptoms of autonomic dysfunction can include presyncope, orthostatic intolerance, chest pain, palpitations, thermoregulatory difficulties, and gastrointestinal complaints and are commonly reported in patients with HSD/hEDS.
“Symptoms of autonomic dysfunction can include presyncope, orthostatic intolerance, chest pain, palpitations, thermoregulatory difficulties, and gastrointestinal complaints and are commonly reported in patients with HSD/hEDS. 63 , 64 , 65”
From Hypermobile Ehlers–Danlos syndrome and disorders of the gastrointestinal tract: What the gastroenterologist needs to know (2022) · cites this paper as [63]
Gazit and colleagues reported increased sensitivity to isoproterenol in people with joint hypermobility.
“The data from Gazit and colleagues describe an increased sensitivity to isoproterenol among those with JH.”
From Postural tachycardia syndrome and other forms of orthostatic intolerance in Ehlers-Danlos syndrome (2018) · cites this paper as Gazit et al., 2003
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- Authors
- Gazit, Y., Nahir, A. M., Grahame, R., & Jacob, G.
- Affiliations
- Technion – Israel Institute of Technology (IL)
- University College London (GB)
- Corresponding author
- Jacob, Giris
- Venue
- The American Journal of Medicine
- Publisher
- Elsevier BV
- Published
- 2003-07
- Volume
- 115
- Issue
- 1
- Pages
- 33-40
- ISSN
- 0002-9343 (ISSN-L)
- OpenAlex
- W2022790525
- Cited by
- 390
- References
- 53
- Citations by year
- 2026: 9 · 2025: 18 · 2024: 17 · 2023: 15 · 2022: 14 · 2021: 20 · 2020: 21 · 2019: 21 · 2018: 24 · 2017: 29 · 2016: 21 · 2015: 25 · 2014: 25 · 2013: 22 · 2012: 12
- FWCI
- 9.91
- Type
- journal article
- Language
- en
- Open access
- closed
- Locations
- The American Journal of Medicine (publishedVersion)
- PubMed (publishedVersion)
- 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 › Cardiology and Cardiovascular Medicine › Heart Rate Variability and Autonomic Control
- Keywords
- joint hypermobility syndrome, autonomic dysfunction, autonomic nervous system, orthostatic intolerance, postural orthostatic tachycardia syndrome, orthostatic hypotension, catecholamine levels, beta-adrenergic responsiveness, extra-articular manifestations, syncope, hyperventilation
- MeSH terms
- Adolescent, Adult, Aged, Arrhythmias, Cardiac, Autonomic Nervous System Diseases, Blood Pressure, Catecholamines, Female, Humans, Joint Instability, Male, Middle Aged, Surveys and Questionnaires, Severity of Illness Index, Body Mass Index
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