EDS “Types” Comprise An Arthritis-Adrenaline Disorder Spectrum

Joint-tissue laxity leads to “fight-or-flight” stimulation. Lax joints and tissue in EDS lead not only to osteoarthritis and injury but also to distensible skin and blood vessels that allow pooling of blood in the lower body (evident in many when their feet turn purple upon standing). The resulting depletion of head circulation with reactive adrenergic stimulation (autonomic imbalance or dysautonomia) adds tachycardia, anxiety, and chronic fatigue to the varicosities and pelvic congestion (with menorrhagia), dizziness on standing (orthostatic hypotension), and “brain fog” caused by lower body blood pooling. This “sympathetic” stimulation (ironic indeed) not only produces postural orthostatic tachycardia syndrome (POTS) but also its  immune/allergic counterpart: Mast cell activation disorder (MCAD) with reactive eyes, skin, airways, and gut. Stimulating the autonomic yin (adrenaline) suppresses its parasympathetic yang, the cholinergic “relax and digest” pathways, to cause irritable bowel syndrome (IBS--constipation/diarrhea/reflux/gall bladder issues from low bowel motility). Because these many autonomic complications are so often attributed to psychiatric disease or stress reactions, I have proposed the term “arthritis-adrenaline disorder” as a subsuming category and preliminary diagnosis for EDS and its variations. The AAD finding pattern1 is shared by all EDS types and many other connective tissue dysplasias (CTD); it can be an icon for recognition and treatment by generalists and a preliminary diagnosis for specialists until further studies define a specific EDS type or CTD.

​The left figure below shows the 80 history and 40 physical findings established as typical by outpatient evaluation of 946 patients for EDS in my genetics clinic from 2011 to 2016. The right figure shows the numbers of history and physical findings from 710 subsequent EDS evaluations (2016-2018) using standard forms with these findings, the history forms filled out by patients beforehand and confirmed by me, the physical forms used by me to standardize my physical examination.1 After completion of the history and physical, patients were given preliminary diagnoses of hypermobile hEDS (velvety skin, marked hypermobility with joint instability), classical cEDS (unusual scarring, less hypermobility), or benign joint hypermobility (BJH-hypermobility with milder joint-skin findings). Figures on the right show distributions of the findings in patients with various preliminary diagnoses, showing overlap and prevalence of tissue-joint laxity and dysautonomia findings in these groups. Scores for the 114 men averaged 30% lower than those for the 596 women, the 59 patients diagnosed with other diseases or primary dysautonomia are not shown. I have seen only 2 patients with vascular EDS, none in this group (after 2011). Similar score distributions have been obtained for ~150 patients evaluated online (>2018).​ From ​https://www.scirp.org/journal/PaperInformation.aspx?PaperID=93210