Surgery

Postural Orthostatic Tachycardia Syndrome

Dermatology

Exercise intolerance

Ehlers–Danlos syndrome

Dysautonomia

Heart failure

Blood pressure

Physical therapy

Internal medicine

Joint hypermobility

Orthostatic vital signs

Medicine

Orthostatic intolerance

Disease

Autonomic Neuroscience

ObjectiveTo review the association between orthostatic intolerance syndromes and both joint hypermobility and Ehlers-Danlos syndrome, and to propose reasons for identifying hereditary connective tissue disorders in those with orthostatic intolerance in the context of both clinical care and research.MethodsWe searched the published peer-reviewed medical literature for papers reporting an association between joint hypermobility or Ehlers-Danlos syndrome and orthostatic intolerance.ResultsWe identified 10 relevant papers. Although methodological variability between studies introduces some limitations, the published literature consistently identifies a significantly higher prevalence of orthostatic intolerance symptoms in patients with joint hypermobility or Ehlers-Danlos syndrome than in healthy controls, and a significantly higher prevalence of cardiovascular and autonomic abnormalities both at rest and during orthostatic challenge. Postural tachycardia syndrome is the most commonly recognized circulatory disorder. The severity of orthostatic symptoms in those with EDS correlates with impairments in quality of life.ConclusionThere is a strong association between several forms of cardiovascular dysfunction, most notably postural tachycardia syndrome, and joint hypermobility or Ehlers-Danlos syndrome. We propose that recognition of joint hypermobility and Ehlers-Danlos syndrome among those with orthostatic intolerance syndromes has the potential to improve clinical care and the validity of research findings.

Postural tachycardia syndrome and other forms of orthostatic intolerance in Ehlers-Danlos syndrome