There is a definite association between EDS/joint hypermobility and dysautonomia as well as MCAS. These syndromes have been linked in epidemiologic surveys although the mechanisms by which they are connected are still being elucidated. Perhaps the underlying connection is through the mast cells releasing various mediators at inappropriate times. Perhaps the linkage, at least in some patients, will turn out to be autoimmune activity. The search and research is ongoing. The following article references these connections. The questionnaires that are mentioned in the abstract can be found on my site here under “USEFUL QUESTIONNAIRES”.
Autonomic symptom burden in the hypermobility type of Ehlers-Danlos syndrome: a comparative study with two other EDS types, fibromyalgia, and healthy controls.
Abstract
AIM:
This study provides insight into the profile and importance of autonomic symptoms in the hypermobility type (HT) of Ehlers-Danlos syndrome (EDS). The impact of these symptoms is put into perspective by comparing with fibromyalgia (FM) and two other EDS types.
METHODS:
Overall, 80 patients with EDS-HT participated, as well as 11 with classical EDS (cEDS), seven with vascular EDS (vEDS), 38 with FM, and 43 healthy controls. All participants filled out the autonomic symptom profile (ASP). Furthermore, they were inquired about quality of life (QOL, SF-36) and factors contributing to the EDS disease burden, e.g., hypermobility (5-point questionnaire, GHQ), fatigue (checklist individual strength, CIS), pain (pain detect questionnaire, PDQ), affective distress (hospital anxiety and depression scale, HADS), and physical activity (Baecke).
RESULTS:
The total autonomic symptom burden was higher in EDS-HT (57.9 ± 21.57) than in controls (11.3 ± 19.22), cEDS (32.3 ± 19.47), and vEDS (29.1 ± 19.18), but comparable to FM (53.8 ± 19.85). Especially orthostatic and gastrointestinal complaints were prevalent. The importance of autonomic symptoms in EDS-HT was emphasized by the correlation with lowered QOL (r = -0.402), fatigue (r = 0.304), and pain severity (r = 0.370). Although affective distress and decreased physical activity are often suggested as possible causes for dysautonomia, the ASP did not correlate with the HADS and Baecke score. By contrast, the correlation of the GHQ (r = 0.298) and PDQ (r = 0.413) with the ASP supports the hypothesis that joint hypermobility and neuropathy may play a role in the development of autonomic symptoms.
CONCLUSION:
Autonomic symptoms, especially orthostatic and gastrointestinal complaints, are frequent extraarticular manifestations of EDS-HT and contribute to the disease burden.
If you are known to have EDS/joint hypermobility you should be screened for the possibly associated conditons including but not limited to POTS, MCAS (mast cell activation syndrome), fibromyalgia and others. If you have been diagnosed with POTS or any of the other syndromes then you should at least consider the possibility that you have Ehlers-Danlos Syndrome as well.
