Background/Purpose: Hypermobility spectrum disorders (HSD) and hypermobile Ehlers Danlos syndrome (hEDS) are among the conditions associated with generalized joint hypermobility (GJH). Alongside myofascial and skeletal pain manifestations, other polymorphic co-morbid manifestations, often multiple, are linked to an autonomic dysregulation syndrome. The aim of this study was to characterize the impact of dysautonomia in patients suffering from joint hypermobility syndrome (HSD/hEDS).

Methods: Incident patients were recruited from consultations at the Center of Competence for Rare Diseases of Non-Vascular EDS. Inclusion criteria were GJH and clinical criteria for hEDS or HSD. Scores on the following questionnaires were collected : SCOPA-AUT (dysautonomia), Nijmegen (hyperventilation), HAD (anxiety, depression), Tampa (kinesiophobia), PSQI (sleep), Gastro-Intestinal Quality of Life Index (GIQLI), Pichot (fatigue), VAS physical pain and VAS psychological pain, VAS quality of life. A comparison of the 2 hEDS and HSD groups was carried out, followed by a principal component analysis (PCA). The search of homogeneous groups of patients was carried out using hierachical cluster analysis.

Results: 36 patients were included (18 hEDS, 18 HSD) with a significantly higher level of anxiety-depression in the hEDS group (25.33 vs 18.39, p = 0.017). The Nijmegen score was higher in the hEDS group (46.91 vs 38.94, p = 0.016). The mean SCOPA-AUT score was 37.08 ± 9.86 and did not differ significantly between the 2 groups; there was no statistically significant difference between the 2 groups for the other parameters studied. The level of dysautonomia was correlated with younger age, high Nijmegen score, kinesiophobia, fatigue, sleep disturbance, marked anxiety-depressive syndrome and poor GI QoL. PCA revealed a strong correlation between the level of dysautonomia and GI QoL. Two homogeneous groups of patients were identified : one of which (n = 23)  was characterized by very high impairment of GI QoL and a high level of dysautonomia.

Conclusion: Our study illustrates the importance of dysautonomia as a major comorbidity in HSD/hEDS. Dysfunctional breathing seems almost constant, and dysautonomia is associated with a major impairment of GI QoL. An anxiety or depressive disorder is very common in these situations, and supports the recently put forward hypothesis of a neuroconnective phenotype. The polyvagal theory provides important physiological clues for understanding these pathologies and for their therapeutic management.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/the-burden-of-dysautonomia-in-patients-suffering-from-pathologies-associated-with-generalized-joint-hypermobility/