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

Autonomic dysfunction can present in many ways.  Patients complained of orthostatic symptoms like fatigue, difficulty concentrating, brain fog, chest pain, palpitations, headache, visual disturbances, shortness of breath and feeling “absent.” Common GI symptoms were early satiety, bloating, nausea and vomiting (particularly after a meal), colonic spasms, constipation and diarrhea.  Sweating too much or too little and dry eyes and mouth were reported.  Raynaud’s phenomenon and purple limbs upon standing affected many HEDS patients.  Sensitivity to light and difficulty focusing vision occurred due to dysregulation of pupils. Urine retention, failure to empty bladder while urinating, and incontinence of urine were also common.

Some symptoms were strongly associated with others.  Fatigue and difficulty in concentrating was most often seen in association with symptoms that affected blood vessels changing size, symptoms that affected secretion by glands, and GI or sleep symptoms.  An overall large burden of autonomic symptoms was also seen in patients with fatigue and difficulty concentration.  Greater concentration difficulties also correlated with worse orthostatic symptoms, bladder symptoms, gastroparesis, dysregulation of pupil motion and an overall large burden of autonomic symptoms.

Many autonomic symptoms (but not those affecting motion of blood vessels or fainting) were correlated to neuropathic pain.  Orthostatic, GI, bladder, pupil and gland secretion symptoms, sleep dysfunction, and overall high autonomic burden were linked to pain severity. Tachycardia when upright and dysautonomia generally were related to severity of pain.

Dysautonomia symptoms were often seen in HEDS, particularly orthostatic and GI symptoms.  Dysautonomia symptoms greatly impacted quality of life and were associated with more fatigue and pain. Dysautonomia was much worse in HEDS than in CEDS, VEDS or fibromyalgia. In particular, orthostatic intolerance dramatically affected quality f life.  The physical limitations observed in POTS patients, the most common form of orthostatic intolerance for HEDS patients, were comparable to those seen in people with congestive heart failure or COPD.  These symptoms contribute to the lower quality of life seen in HEDS patients when compared to other EDS patients.

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.