Hypermobility Type Ehlers Danlos Syndrome and Autonomic Dysfunction (Part Two)

The Autonomic Symptom Profile (ASP) is a questionnaire that assesses symptoms in functional areas controlled by the autonomic nervous system.  The autonomic nervous system regulates many involuntary functions, such as heart rate, blood pressure, urination and digestion.  Dysfunction of the autonomic nervous system can affect many organ systems.

One study evaluated Ehlers Danlos patients for autonomic symptoms using the ASP.  The patients in this study had classic Ehlers Danlos (cEDS), vascular Ehlers Danlos (vEDS) or hypermobility type Ehlers Danlos (HEDS).  Symptom burden was compared among these different presentations of EDS and to healthy controls.

Patients with HEDS had the highest total burden of autonomic symptoms among EDS patients.  All EDS patients had more autonomic symptoms than healthy controls.  HEDS caused more orthostatic symptoms (symptoms that happen when standing up) than in other EDS forms.  94% of HEDS patients had orthostatic symptoms, including lightheadedness, dizziness, palpitations, nausea, blurred vision and anxiety.  Though many patients said they often “felt faint”, true fainting was not common.  These symptoms could be provoked or worsened with physical activity, heat, meals, or change in position.

Patients with HEDS also had the highest burden of GI symptoms compared to other types of EDS.  73% had gastroparesis, 66% had chronic constipation, and 64% had regular diarrhea.  Diarrhea was found to be the most impairing GI symptom.

HEDS patients had a larger impact of orthostatic symptoms and bladder dysfunction than either CEDS or VEDS.  Compared to just CEDS, HEDS showed more GI, secretomotor (release of fluid by glands) and pupillomotor (motion of pupil) symptoms.  Compared to just VEDS, HEDS patients had more vasomotor burden (symptoms related to the dilation of blood vessels).  HEDS autonomic burden was similar to those seen in fibromyalgia patients, with more bladder dysfunction and less sleep dysfunction.

Higher autonomic symptom burden was associated with more physical impairment, pain and decreased vitality.  More hypermobility was associated with higher burden of orthostatic symptoms, GI symptoms generally, gastroparesis, diarrhea, vasomotor symptoms and overall autonomic symptom burden.

References:

de Wandele I, et al. Dysautonomia and its underlying mechanisms in the hypermobility type of Ehlers-Danlos syndrome. Seminars in Arthritis and Rheumatism 2014, 44: 93-100.

de Wandele I, et al. Autonomic symptom burden in the hypermobility type of Ehlers-Danlos syndrome: A comparative study with two other EDS types, fibromyalgia, and healthy controls. Seminars in Arthritis and Rheumatism 2014, 44: 353-361.

Wallman D, et al. Ehlers-Danlos Syndrome and Postural Tachycardia Syndrome: A relationship study. Journal of Neurological Sciences 2014, 340: 99-102.