Pain in Ehlers-Danlos Syndrome Is Common, Severe, and Associated with Functional Impairment
Open accessAbstract
Context The Ehlers-Danlos Syndrome (EDS) is a clinically and genetically heterogeneous group of heritable connective tissue disorders characterized by joint hypermobility, skin hyperextensibility, and tissue fragility. Musculoskeletal pain is mentioned in the diagnostic criteria and described as early in onset, chronic, and debilitating. However, systematic research on pain in EDS is scarce.Objectives We investigated prevalence and impact of pain and associated features in a large group of EDS patients.Methods We performed a study among members of the Dutch EDS patient organization (n=273) and included the McGill Pain Questionnaire to investigate various aspects of pain, the Sickness Impact Profile to study functional impairment, the Symptom Checklist subscale sleep to evaluate sleep disturbances, and the Checklist Individual Strength subscale fatigue to determine fatigue severity.Results The results of this study show that 1) chronic pain in EDS is highly prevalent and associated with regular use of analgesics; 2) pain is more prevalent and more severe in the hypermobility type than in the classic type; 3) pain severity is correlated with hypermobility, dislocations, and previous surgery; 4) pain is correlated with low nocturnal sleep quality; and 5) pain contributes to functional impairment in daily life, independent of the level of fatigue.Conclusion From this large cohort of EDS patients, we conclude that pain is common and severe in EDS. Pain is related to hypermobility, dislocations, and previous surgery and associated with moderate to severe impairment in daily functioning. Therefore, treatment of pain should be a prominent aspect of symptomatic management of EDS.
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hEDS is associated with significant somatic complaints related to musculoskeletal manifestations: fibromyalgia (40%), chronic fatigue (38%), and pain (almost 100%).
“hEDS is considered to sit on the more severe end of the spectrum as it is associated with significant somatic complaints related to musculoskeletal manifestations such fibromyalgia (40%), chronic fatigue (38%), and pain (almost 100%) (acute and chronic, nociceptive, neuropathic, and nociplastic).”
From Hypermobile Ehlers–Danlos syndrome and disorders of the gastrointestinal tract: What the gastroenterologist needs to know (2022) · cites this paper as [11]
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- Authors
- Voermans, N. C., Knoop, H., Bleijenberg, G., & van Engelen, B. G.
- Affiliations
- Radboud University Medical Center (NL)
- Corresponding author
- Voermans, Nicol C.
- Venue
- Journal of Pain and Symptom Management
- Publisher
- Elsevier BV
- Published
- 2010-09
- Volume
- 40
- Issue
- 3
- Pages
- 370-378
- ISSN
- 0885-3924 (ISSN-L)
- OpenAlex
- W2152518735
- Cited by
- 237
- References
- 26
- Citations by year
- 2026: 11 · 2025: 9 · 2024: 15 · 2023: 15 · 2022: 20 · 2021: 28 · 2020: 17 · 2019: 16 · 2018: 10 · 2017: 12 · 2016: 17 · 2015: 15 · 2014: 13 · 2013: 11 · 2012: 11
- FWCI
- 7.02
- Type
- journal article
- Language
- en
- Open access
- bronze
- doi.org
- Licence
- Locations
- Journal of Pain and Symptom Management (publishedVersion)
- PubMed (publishedVersion)
- Radboud Repository (Radboud University) (submittedVersion)
- Topics
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Genetics › Connective tissue disorders research
- Health Sciences › Medicine › Pharmacology › Musculoskeletal pain and rehabilitation
- Health Sciences › Medicine › Pediatrics, Perinatology and Child Health › Pediatric Pain Management Techniques
- Keywords
- Ehlers-Danlos syndrome, chronic pain, pain prevalence, pain intensity, functional disability, joint hypermobility, dislocations, previous surgery, sleep disorders, fatigue, analgesic use, hereditary connective tissue disorders, McGill Pain Questionnaire, Sickness Impact Profile, Checklist Individual Strength
- MeSH terms
- Activities of Daily Living, Adolescent, Adult, Aged, Aged, 80 and over, Analgesics, Cross-Sectional Studies, Ehlers-Danlos Syndrome, Fatigue, Female, Humans, Male, Middle Aged, Pain, Pain Measurement, Sleep Wake Disorders, Young Adult
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