EDS Research Index
An independent, source-linked index of what is known — and what is still being worked out — about the Ehlers-Danlos syndromes. Every record names its evidence, its stratum, its subtype scope, and its diagnostic era.
Five strata of evidence
Rare-disease knowledge does not live in one place. This index files every source under one of five strata and never collapses them into a single score — a forum pattern and a randomized trial are both real evidence, and they are not the same kind of real.
clinical
Peer-reviewed literature, trials, guidelines, and consensus statements.
community
Venue-level patterns from forums, support groups, and patient organizations — never individual posters.
historical
Pre-nosology case descriptions, performer-era records, and documented folk management.
registry
Patient registries, trial registrations, and rare-disease reference rails.
gray literature
Preprints, theses, and working papers ahead of peer review.
Browse the index
Subtypes
All thirteen 2017-classification types plus HSD — genes, inheritance, prevalence, and what distinguishes each.
Timeline
From van Meek'ren's 1682 report through Berlin, Villefranche, and the 2017 International Classification to KLK15.
Practices
Management and treatment records — clinical, community-reported, and folk — with evidence tier and risk made explicit.
Community knowledge
Venue-level signals from forums and support groups, filed as reports — with the venues named and individuals never.
Source catalog
Every cited source with its stratum, tier, publisher, and stable identifier.
Research program
Discovery monitors, open questions, collections, and the append-only run ledger.
Methodology
The full research methodology: strata, tiers, corroboration, criteria eras, review lifecycle, and publication policy.
Data
The open YAML corpus behind every page — downloadable, diffable, auditable.
Cross-stratum convergences
Records where independent strata agree — the pattern a rare-disease index exists to surface. The lidocaine record is the reference case: patients reported anesthetic failure for decades before a randomized trial confirmed it.
Local anesthetic resistance — the index's reference convergence
establishedpracticestrata convergecautionPatients reported local-anesthetic failure for decades. Hakim and Grahame's 2005 survey found 58% of hypermobile patients reported inadequate anesthesia vs 21% of controls; a 2019 survey (n=988) found 88% vs 33%; in 2025 a randomized cross-over trial (n=135) confirmed shorter lidocaine duration in EDS patients. The arc — forum reports, structured survey, randomized confirmation — is the model this index exists to document.
Diagnosis navigation and specialist-finding strategies
community signalcommunity signalstrata convergeCommunity venues maintain informal routing — which specialists diagnose, which tests matter, how to prepare a diagnostic case. This is the community's response to the documented multi-year diagnostic delay, not a clinical pathway.
The "trifecta" — hEDS, POTS/dysautonomia, and MCAS co-clustering
community signalcommunity signalstrata convergePatient communities converged on the hEDS–POTS–MCAS triad years before it appeared in clinical reviews. Registry data now supports the clustering: in the Global Registry survey (n=505), POTS and mast cell activation syndrome were among the alternative diagnoses participants most endorsed as accurate. The triad is community-coined and increasingly clinically studied; the mechanism remains unexplained.
hEDS patients average ten alternative diagnoses before confirmation
establishedfindingstrata convergeThe Global Registry survey (n=505, clinically confirmed hEDS) found a mean of 10.45 alternative diagnoses — most commonly anxiety, depression, and migraine — with functional neurological disorder and multiple sclerosis among the most-rejected labels. Post-diagnosis, participants most endorsed POTS, cervical instability, and MCAS as accurate.
Craniocervical instability and tethered cord — the contested frontier
contestedfindingstrata convergeCraniocervical instability and tethered-cord claims circulate strongly in community venues and some surgical case series, and CCI was among the most-endorsed diagnoses in the registry survey. Controlled evidence remains limited and surgical intervention carries real risk; this index marks the area contested rather than dismissing it.
The 2017 hEDS criteria require hypermobility, systemic manifestations, and exclusion
establishedfindingstrata convergehEDS diagnosis under the 2017 International Classification requires (1) generalized joint hypermobility by Beighton score, (2) two or more of systemic manifestations, positive family history, or musculoskeletal complications, and (3) exclusion of other connective-tissue disorders. Unlike every other subtype, hEDS has no molecular confirmation test.
Categories
Disease historiography
How the EDS concept formed: first descriptions, performer-era records, nosology revisions, and the modern classification era.
Diagnosis and classification
The criteria machinery — Beighton scoring, the 2017 three-part hEDS criteria, subtype confirmation, and the criteria-era problem.
Genetics and biomarkers
The molecular map — confirmed genes for twelve subtypes, the hEDS search, registries and cohorts.
Comorbidities and systemic features
The multisystem reality — dysautonomia and POTS, MCAS, GI, pain, fatigue, and the comorbidity structure the community named before the literature did.
Management and clinical care
What the clinical literature supports — physiotherapy mainstay, pain management, anesthetic and surgical cautions — with evidence tiers made explicit.
Community knowledge
What patient venues carry that the formal literature still lacks — recurring self-reported signals, practical knowledge, and lived patterns.
Folk practices and early management
Pre-criteria management and folk practices — how laxity was accommodated and treated before the modern evidence era. Documented as historical record, not endorsement.
Patient experience and diagnostic odyssey
The measured human cost — diagnostic delay, dismissal, and the structured record of what getting diagnosed actually takes.
Research programs, registries, and trials
The living research infrastructure — the Global Registry, ECHO, HEDGE, clinical trials, and the institutes carrying EDS science forward.