schema: eds-research/corpus/v1 category: id: comorbidities label: Comorbidities and systemic features description: "Conditions that often come with EDS: dysautonomia and POTS, mast cell activation, digestive problems, pain, and fatigue." order: 40 records: - id: com-trifecta-pattern kind: signal title: "The “trifecta”: hEDS, POTS, and mast cell activation reported together" description: Patient communities often describe hEDS, POTS, and mast cell activation syndrome occurring together, and call the combination the “trifecta”. summary: Patient communities often describe hEDS, POTS (a form of dysautonomia), and mast cell activation syndrome (MCAS) occurring together, and call the combination the “trifecta”. In a Global Registry survey of 505 people with hEDS, POTS and MCAS were among the diagnoses participants most often endorsed as accurate. Whether the three share a mechanism is unknown. subtypes: [heds, hsd] status: community-signal criteria_era: international-2017 evidence: - stratum: community tier: cross-venue-pattern source_ids: [source-ec36295ad04f34148c63, source-641ce29242a70e8a5f01] note: Recurring across Reddit and Inspire over multiple years. - stratum: registry tier: registry-analysis source_ids: [source-9a67b455782a95159bd9] note: POTS and MCAS were among the diagnoses participants most often endorsed as accurate. corroboration: convergent venues: [venue-reddit-ehlersdanlos, venue-inspire-eds-hsd] reviewed_at: "2026-09-16" reassess_by: "2026-12-16" tags: [dysautonomia, mcas, trifecta] - id: com-multisystem-2017 kind: finding title: The 2017 classification papers cover symptoms beyond joints and skin description: The 2017 hEDS criteria count connective-tissue signs such as hernias and prolapse. Papers published with them also review pain and other symptoms. summary: "The hEDS criteria in the 2017 classification count signs of a more generalized connective tissue disorder, such as soft skin, hernias, pelvic floor prolapse, mitral valve prolapse, and aortic root dilatation. Chronic musculoskeletal pain counts toward the criteria as a musculoskeletal complication (Feature C), but digestive, autonomic, and fatigue problems are not diagnostic criteria. Papers published alongside the classification describe them: Tinkle and colleagues review the symptoms and disorders reported with hEDS, and Chopra and colleagues review chronic pain in EDS." date: "2017-03" date_precision: month subtypes: [all-eds] status: established criteria_era: international-2017 evidence: - stratum: clinical tier: consensus-statement source_ids: [source-36ad1cab53c4cea4f4b4] - stratum: clinical tier: expert-review source_ids: [source-1ad3f0edd57e0fab1524, source-d7ba05272083315d9e1f] reviewed_at: "2026-09-16" tags: [multisystem] - id: com-misdiagnosis-load kind: finding title: In a registry survey, people with hEDS reported about ten other diagnoses summary: A Global Registry survey of 505 people with clinically confirmed hEDS, published in 2023, found an average of 10.45 other diagnoses. Anxiety, depression, and migraine were the most common. Participants most often rejected functional neurological disorder, multiple sclerosis, and fibromyalgia as wrong, and most often endorsed POTS, cervical instability, and MCAS as accurate. date: "2023" date_precision: year subtypes: [heds] status: probable criteria_era: international-2017 evidence: - stratum: registry tier: registry-analysis source_ids: [source-9a67b455782a95159bd9] - stratum: clinical tier: qualitative-study source_ids: [source-21337422dde3976b584c] note: A 2025 qualitative study describes misdiagnosis and dismissal on the way to an hEDS diagnosis. corroboration: convergent reviewed_at: "2026-09-16" tags: [misdiagnosis, registry] - id: com-cci-tethered-cord kind: finding title: "Craniocervical instability and tethered cord in hEDS: contested" description: Craniocervical instability and tethered cord come up often in hEDS patient communities. Controlled evidence is limited, and surgery carries real risk. summary: Craniocervical instability (CCI) and tethered cord come up often in patient communities, and cervical instability was one of the diagnoses participants most often endorsed as accurate in a Global Registry survey of people with hEDS. Controlled evidence is limited, and surgery for these conditions carries real risk. subtypes: [heds, ceds] status: contested criteria_era: international-2017 evidence: - stratum: community tier: cross-venue-pattern source_ids: [source-ec36295ad04f34148c63, source-641ce29242a70e8a5f01] - stratum: registry tier: registry-analysis source_ids: [source-9a67b455782a95159bd9] note: Cervical instability was among the diagnoses participants most often endorsed as accurate. corroboration: convergent venues: [venue-reddit-ehlersdanlos, venue-inspire-eds-hsd] reviewed_at: "2026-09-16" reassess_by: "2026-12-16" tags: [cci, tethered-cord, contested] - id: com-gi-dysautonomia-cluster kind: finding title: GI dysmotility and autonomic features are documented comorbidity domains description: A 2017 review describes digestive dysmotility and autonomic problems, including POTS, as common comorbidities in hEDS and HSD. summary: The 2017 series and subsequent cohort work document gastrointestinal dysmotility (reflux, gastroparesis, constipation) and autonomic dysfunction (orthostatic intolerance, POTS) as leading comorbidity domains in hEDS/HSD. In a 2025 survey at a German EDS clinic, musculoskeletal pain and joint instability were the most common first symptoms. subtypes: [heds, hsd] status: probable criteria_era: international-2017 evidence: - stratum: clinical tier: expert-review source_ids: [source-1ad3f0edd57e0fab1524] - stratum: clinical tier: cross-sectional-study source_ids: [source-de164233cddfefc4105a] reviewed_at: "2026-09-16" tags: [gi, dysautonomia]