EHLERS-DANLOS SYNDROME & DYSAUTONOMIA

[Pages:52]EHLERS-DANLOS SYNDROME &

DYSAUTONOMIA

Presented By Lauren Stiles, JD President & Co-Founder

New York Institute of Technology December 8, 2017

HOW MANY PEOPLE WITH EDS

HAVE AUTONOMIC DYSFUNCTION?

Most studies are on hEDS

hEDS patients have more autonomic symptoms than other EDS types

49% of hEDS patients have POTS, 31% have OI, 20% have normal hemodynamics1

In hEDS, autonomic dysfunction is classified as:

mild (47%) moderate (33%) Severe (3.3%)2

1 Celletti, C., Camerota, F., Castori, M., et al., 2017. Orthostatic intolerance and postural orthostatic tachycardia syndrome in joint hypermobility syndrome/Ehlers-Danlos syndrome, hypermobility type: Neurovegetative dysregulation or autonomic failure? BioMed Res Int., 9161865. 2 De Wandele, I., Rombaut, L., Leybaert, L., et al., 2014b. Dysautonomia and its underlying mechanisms in the hypermobility type of Ehlers-Danlos syndrome. Semin Arthritis Rheum. 44(1) 93-100.

Disease-Based

Dysautonomias

Syndromic (Possibly Disease Based?)

Central

Peripheral

Multiple System Atrophy

Parkinson's/ Lewy Body Dementias

Pure Autonomic

Failure

Small Fiber Neuropathies

Metabolic

Diabetes

Inflammatory

Immune

Genetic

Mitochondrial

POTS/OI

Complex Regional Pain

Syndrome

Reflex Syncope

Chronic Fatigue Syndrome

Interstitial Cystitis

Fibromyalgia

IBS/CIPO/ Gastroparesis

Chronic Migraine

Adapted from Chelimsky T, Robertson D, Chelimsky G. Disorders of the autonomic nervous system. In Daroff: Bradley's Neurology in Clinical Practice. 6th ed. Philadelphia, PA; Elsevier: chap 77.

POTS: DIAGNOSTIC CRITERIA

HR increase of 30bpm from supine to standing within 10 minutes (40bpm for ages 12-18)

In the absence of orthostatic hypotension (defined as 20/10mmHg drop within 3 min. of standing)

Symptoms of orthostatic intolerance lasting 6 months

Symptoms exacerbated by standing and improved with recumbency

Absence of other overt causes of orthostatic symptoms or tachycardia

POTS SYMPTOMS

Pronounced orthostatic

Sensitivity to heat/cold

tachycardia

Sensitivity to light/sounds

Palpitations

Migraines

Shortness of breath

Profound fatigue

Lightheadedness/pre-syncope Weakness

Syncope (20-30% with

Tremulousness

overlapping NCS)

Exercise intolerance

GI motility (33% too fast, 33% Dependent acrocyanosis

too slow) Nausea Bladder dysfunction (17%)

Flushing Increased allergies

DEPENDENT ACROCYANOSIS

Acrocyanotic legs after a few minutes of standing Prolonged blanching/delayed capillary refill

EPIDEMIOLOGY OF POTS

Common form of orthostatic intolerance US estimates, 500K-3M Newer estimates are in the higher range

For comparison... MS: 400K US estimate Parkinson's: 1M US estimate

Mayo Clinic estimates 1 in 100 teens (~500K) About half of all patients have onset in adulthood

POTS IS NOT A "TEENAGE SYNDROME"

1 Stiles L, Ross A. Physician Patient Interaction in Postural Orthostatic Tachycardia Syndrome. Dysautonomia International. 2014.

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