Pain management in the Ehlers–Danlos syndromes

Chopra, Pradeep; Tinkle, Brad; Hamonet, Claude; Brock, Isabelle; Gompel, Anne; Bulbena, Antonio; Francomano, Clair

doi:10.1002/ajmg.c.31554

Cited by 209Connective tissue disorders researchMusculoskeletal pain and rehabilitationPediatric Pain Management Techniques

Pain management in the Ehlers–Danlos syndromes

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Chopra, Pradeep, Tinkle, Brad, Hamonet, Claude, Brock, Isabelle, Gompel, Anne, Bulbena, Antonio, Francomano, Clair·2017·American Journal of Medical Genetics Part C: Seminars in Medical Genetics

DOI 10.1002/ajmg.c.31554

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Abstract

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.
“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]
The Introduction (page not provided) says pain is common in Ehlers–Danlos syndrome and may correlate with hypermobility. The Methods section also reports pain in 90% of a group of patients with EDS. These passages support the claim for people with EDS, but not for all individuals with hypermobility. (LLM, conf=0.84)
Experimental — accuracy not yet measured
Clinical features of HSD and hEDS include associations with cardiovascular autonomic dysfunction, gastrointestinal difficulties, fatigue, pain syndromes, gynecological and obstetric problems, and mental health concerns.
“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]
Citation (14) is attached to “pain syndromes,” not to the other conditions listed. The paper’s Methods section reports common, varied pain in hEDS, and its Chronic Pain section describes pain as a prevalent symptom of EDS. These passages support the hEDS portion, but they do not establish the association specifically for HSD. (LLM, conf=0.82)
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Research works printed but not linked: 2

Gompel A. 2016. Les manifestations gynecologi- ques du syndrome d'Ehlers-Danlos hyper- mobile. Journal de Readaptaton Medicale 36:64-66.
Hamonet C, Ravaud P, Villeneuve S, Gompel A, Serre N, Fredy D, Deparcy D, Mazaltarine G, Zeitoun JD, Metlaine A, L eger D, Benachi A, Cordier AG, C esaro P, Loche C, Dantchev N, Vienne M, S ejourn e C, Laouar N, Gogly B, Landru MM, Moussa Badran S, Raffray Y, Challe G, Doursou- nian L, Mohler J. 2012. Ehlers-Danlos syndrome (about 664 cases). Statistical analysis of clinical signs from 644 patients with a Beighton scale!4/9. Abstracts book of First International Symposium on the Ehlers-Danlos syndrome, Ghent, Belgium, p. 114.

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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
ORCID: Tinkle, Brad
Hamonet, Claude
Brock, Isabelle
Gompel, Anne
Bulbena, Antonio
Francomano, Clair
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
DOI: 10.1002/ajmg.c.31554
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
Licence: onlinelibrary.wiley.com/termsAndConditions#vor (vor)
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 › 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 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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