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Pain management in the Ehlers–Danlos syndromes
Open accessAbstract
Chronic pain in the Ehlers-Danlos syndromes (EDS) is common and may be severe. According to one study, nearly 90% of patients report some form of chronic pain. Pain, which is often one of the first symptoms to occur, may be widespread or localized to one region such as an arm or a leg. Studies on treatment modalities are few and insufficient to guide management. The following is a discussion of the evidence regarding the underlying mechanisms of pain in EDS. The causes of pain in this condition are multifactorial and include joint subluxations and dislocations, previous surgery, muscle weakness, proprioceptive disorders, and vertebral instability. Affected persons may also present with generalized body pain, fatigue, headaches, gastrointestinal pain, temporomandibular joint pain, dysmenorrhea, and vulvodynia. Pain management strategies may be focused around treating the cause of the pain (e.g., dislocation of a joint, proprioceptive disorder) and minimizing the sensation of pain. Management strategies for chronic pain in EDS includes physical therapy, medications, as well as durable medical equipment such as cushions, compressive garments, and braces. The different modalities are discussed in this paper. © 2017 Wiley Periodicals, Inc.
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“Pain is a common symptom in individuals with hypermobility (14).”
From Joint Hypermobility Links Neurodivergence to Dysautonomia and Pain (2022) · cites this paper as [14]
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“Clinical features of HSD and hEDS are not limited to musculoskeletal and cutaneous symptoms (9). Associations exist with cardiovascular autonomic dysfunction (12), gastrointestinal difficulties (13), fatigue (12) and pain syndromes (14), gynecological and obstetric problems (15, 16), and mental health concerns (17–19).”
From Joint Hypermobility Links Neurodivergence to Dysautonomia and Pain (2022) · cites this paper as [14]
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A framework for the classification of joint hypermobility and related conditions
Castori, M., Tinkle, B., Levy, H., Grahame, R., Malfait, F., & Hakim, A. (2017) · American Journal of Medical Genetics Part C: Seminars in Medical Genetics · doi:10.1002/ajmg.c.31539 · Cited by 602
Self-citationMalfait, F., Castori, M., Francomano, C. A., Giunta, C., Kosho, T., & Byers, P. H. (2020) · Nature Reviews Disease Primers · doi:10.1038/s41572-020-0194-9 · Cited by 346
Self-citationDemmler, J. C., Atkinson, M. D., Reinhold, E. J., Choy, E., Lyons, R. A., & Brophy, S. T. (2019) · BMJ Open · doi:10.1136/bmjopen-2019-031365 · Cited by 271
Gensemer, C., Burks, R., Kautz, S. A., Judge, D. P., Lavallee, M. E., & Norris, R. A. (2020) · Developmental Dynamics · doi:10.1002/dvdy.220 · Cited by 162
Kucharik, A. H., & Chang, C. (2020) · Clinical Reviews in Allergy & Immunology · doi:10.1007/s12016-019-08755-8 · Cited by 122
Metadata
- Authors
- Chopra, P., Tinkle, B., Hamonet, C., Brock, I., Gompel, A., Bulbena, A., & Francomano, C.
- Affiliations
- Universitat Autònoma de Barcelona (ES)
- Corresponding author
- Chopra, Pradeep
- Venue
- American Journal of Medical Genetics Part C: Seminars in Medical Genetics
- Publisher
- Wiley
- Published
- 2017-02-10
- Volume
- 175
- Issue
- 1
- Pages
- 212-219
- ISSN
- 1552-4868 (ISSN-L)
- 1552-4876
- OpenAlex
- W2586740513
- Cited by
- 209
- References
- 95
- Citations by year
- 2026: 9 · 2025: 25 · 2024: 36 · 2023: 26 · 2022: 18 · 2021: 37 · 2020: 20 · 2019: 21 · 2018: 12 · 2017: 4
- FWCI
- 2.00
- Type
- journal article
- Language
- en
- Open access
- bronze
- doi.org
- Locations
- American Journal of Medical Genetics Part C Seminars in Medical Genetics (publishedVersion)
- PubMed (publishedVersion)
- Topics
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Genetics › Connective tissue disorders research
- Health Sciences › Health Professions › Physical Therapy, Sports Therapy and Rehabilitation › Musculoskeletal pain and rehabilitation
- Health Sciences › Medicine › Pediatrics, Perinatology and Child Health › Pediatric Pain Management Techniques
- Keywords
- Ehlers-Danlos syndrome, chronic pain, pain management, joint hypermobility, vertebral instability, muscle weakness, temporomandibular joint pain, dysmenorrhea, vulvodynia, physiotherapy, analgesics, orthotic support
- MeSH terms
- Combined Modality Therapy, Ehlers-Danlos Syndrome, Humans, Pain Management
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