Key result
Among adults with hypermobile Ehlers-Danlos syndrome or hypermobility spectrum disorder, 48% had comorbid postural orthostatic tachycardia syndrome, which was associated with significantly higher resting baroreflex sensitivity compared to those without POTS.
Why the study?
Joint hypermobility and hypermobile Ehlers-Danlos syndrome are associated with orthostatic intolerance, but autonomic profiles had not been explored using a multimodal approach in a large cohort.
Observational (n=102)
Abnormal autonomic regulation, particularly POTS, is highly prevalent in adults with hEDS/HSD, and resting baroreflex sensitivity may help distinguish POTS from other autonomic profiles.
May support POTS screening in hypermobility disorders; leaves open causal role of baroreflex sensitivity.
There is an association between joint hypermobility, hypermobile Ehlers-Danlos syndrome (hEDS) and different forms of orthostatic intolerance. Objective: to explore autonomic profile in a large cohort of adults with hEDS and hypermobility spectrum disorder (hEDS/HSD) with a multimodal approach. In this observational retrospective study, heart rate, blood pressure and baroreflex sensitivity were estimated in 102 hEDS/HSD subjects during deep breathing, Valsalva maneuver, standing up: 30-15 ratio, Head-Up Tilt and sustained handgrip. Abnormal results and head-up tilt test were common and included postural orthostatic tachycardia syndrome (POTS; 48%), orthostatic intolerance (25.5%) and hypotension (3.9%). Baroreflex sensitivity was significantly different in individuals with POTS compared to the others. This study confirms the high rate and heterogeneity of abnormal autonomic regulation in hEDS/HSD, and suggests the baroreflex sensitivity might distinguish comorbid POTS from other profiles in this subgroup of patients. Abnormal autonomic regulation is common in adults with hEDS/HSD and should be regularly assessed for tailoring the management approach.
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Celletti et al. (2020) conducted an observational in Hypermobile Ehlers-Danlos Syndrome / Hypermobility Spectrum Disorder (n=102). Postural orthostatic tachycardia syndrome (POTS) vs. hEDS/HSD without POTS was evaluated on Resting baroreflex sensitivity (BRS) (p=<0.03). Among adults with hypermobile Ehlers-Danlos syndrome or hypermobility spectrum disorder, 48% had comorbid postural orthostatic tachycardia syndrome, which was associated with significantly higher resting baroreflex sensitivity compared to those without POTS.
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