Key result
Generalized hypermobility spectrum disorder links to 43% POTS prevalence, exceeding hypermobile EDS and controls.
Why the study?
It remains unclear whether orthostatic intolerance is a comorbidity or a fundamental part of the pathophysiology of G-HSD or hEDS.
Does the prevalence of orthostatic intolerance differ among young women with G-HSD, hEDS, and healthy controls?
Cross-Sectional (n=45)
No
Does the prevalence of orthostatic intolerance differ among young women with G-HSD, hEDS, and healthy controls?
Young women with hypermobility spectrum disorders experience significant orthostatic intolerance and autonomic dysfunction that negatively impacts quality of life, though POTS prevalence varies by testing method.
Higher POTS prevalence in G-HSD warrants subtype-aware evaluation; leaves open prospective validation before practice change.
Orthostatic intolerance (OI) is frequently reported in young women with generalized hypermobility spectrum disorder (G-HSD) and hypermobile EDS (hEDS). However, it remains currently unclear whether OI is a comorbidity or fundamental part of the pathophysiology of G-HSD or hEDS. This study investigated the prevalence and impact of OI in young women across the hypermobility spectrum. Forty-five women (14-30 years, 15 controls, 15 G-HSD, and 15 hEDS) undertook a head-up tilt (HUT) and active stand test. Postural Orthostatic Tachycardia Syndrome (POTS) and Orthostatic Hypotension (OH) were assessed using age-related criteria. Autonomic dysfunction and quality-of-life questionnaires were also completed. The prevalence of POTS was higher in women with G-HSD than hEDS and control groups during HUT (43% vs. 7% and 7%, respectively, p < 0.05), but similar between groups during the active stand (47%, 27%, and 13% for G-HSD, hEDS, and control, respectively). No participants had OH. hEDS and G-HSD participants reported more severe orthostatic symptoms and poorer quality of life than controls. Although POTS was observed in hypermobile participants, there is no conclusive evidence that its prevalence differed between groups due to differences between the HUT and active stand assessments. Nevertheless, OI and broader autonomic dysfunction impacted on their quality of life.
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Peebles et al. (2022) conducted a cross-sectional in Generalized hypermobility spectrum disorder (G-HSD) and hypermobile Ehlers-Danlos syndrome (hEDS) (n=45). Head-up tilt (HUT) and active stand test vs. Healthy controls was evaluated on Prevalence of Postural Orthostatic Tachycardia Syndrome (POTS) during head-up tilt (HUT) (p=0.04). The prevalence of Postural Orthostatic Tachycardia Syndrome during head-up tilt was significantly higher in young women with generalized hypermobility spectrum disorder (43%) compared to those with hypermobile EDS (7%) and healthy controls (7%).
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