Management of chronic pain in Ehlers–Danlos syndrome: Two case reports and a review of literature
Open accessAbstract
Rationale: Ehlers–Danlos syndromes (EDSs) are a heterogeneous group of heritable connective tissue disorders involving defective collagen synthesis. Patients with EDS are prone for chronic myofascial pain, apart from other comorbidities. Although the initial pathology is commonly nociceptive, progression of EDS leads to neuropathies and central sensitization of pain signals. Overall, approximately 90% or more EDS patients suffer from chronic pain. There are no existing guidelines to manage chronic pain in EDS patients. In this article, we illustrate 2 case reports of EDS patients with chronic pain and review the relevant literature regarding the pathological considerations and management of chronic pain in EDS. Patient concerns: Our first patient had worsening multiple joint pains despite treatment with medications and a recent diagnosis of EDS-type 3. The second patient had a complicated history including longstanding EDS and multiple previous surgeries. She was referred due to concern about complex regional pain syndrome (CRPS) in her upper extremity. Diagnoses: For the first patient, apart from chronic pain due to her EDS, her history also revealed that she suffered from kinesiophobia. For the second patient, a detailed clinical assessment revealed no signs of CRPS but instead a generalized worsening of EDS symptoms. Interventions: Both patients were evaluated by the multidisciplinary team consisting of a pain physician, nurse, pain psychologist, and a kinesiologist. Their treatment involved optimization of daily medications, participation in pain self-management sessions that included cognitive behavioral therapy, graded exercises, coping, and relaxation strategies. Outcomes: Both patients demonstrated significantly better pain control and achieved overall improvement in daily living. Lessons: EDS is a complex illness with a multitude of symptoms. As in our patients, EDS patients also suffer from panic and anxiety disorders that increase the burden of pain. Apart from optimization of medications, EDS patients are best managed by a multidisciplinary approach that includes pain education and life style changes. The caution that they are resistant to local anesthetics is unwarranted. Appropriate pain interventions in selected patients may have a role to decrease pain intensity.
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Zhou, Rewari, and Shanthanna's two case reports investigated a 6-session pain management program for individuals with EDS.
“Zhou, Rewari, and Shanthanna’s two case reports [52] investigated a 6-session pain management program for individuals with EDS.”
From Psychological interventions for individuals with Ehlers-Danlos syndrome and hypermobility spectrum disorder: a scoping review (2023) · cites this paper as [52]
The Case 2 pain-clinic-management section describes a self-management group with six dedicated sessions. The Abstract (Interventions) says both patients participated in pain self-management sessions, but the Case 1 account does not establish that the first patient attended the six-session program. Our copy therefore supports the two-case-report description, but not the six-session detail for both patients. (LLM, conf=0.85)
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Sorted by citations
Song, J. Z., Luong, D., Feldman, E. C. H., Tran, S., Perrier, L., Eubanks, K., Bayley, M., Kastner, M., Slepian, M., & Munce, S. E. P. (2023) · Orphanet Journal of Rare Diseases · doi:10.1186/s13023-023-02799-y · Cited by 9 · see citing works
- Authors
- Zhou, Z., Rewari, A., & Shanthanna, H.
- Affiliations
- Michael DeGroote School of Medicine
- Department of Anesthesia
- McMaster University (CA)
- Corresponding author
- Shanthanna, Harsha
- Venue
- Medicine
- Published
- 2018-11
- Volume
- 97
- Issue
- 45
- Pages
- e13115
- ISSN
- 0025-7974 (ISSN-L)
- 1536-5964
- PMC
- PMC6250522
- OpenAlex
- W2900377812
- Cited by
- 46
- References
- 38
- Citations by year
- 2026: 6 · 2025: 3 · 2024: 10 · 2023: 7 · 2022: 9 · 2021: 4 · 2020: 7
- FWCI
- 1.77
- Type
- journal article
- Language
- en
- Open access
- gold
- doi.org
- Licence
- Locations
- Medicine (publishedVersion, cc-by-nc)
- Europe PMC (PubMed Central) (publishedVersion, cc-by-nc)
- PubMed Central (publishedVersion, cc-by-nc)
- PubMed (publishedVersion)
- Topics
- Health Sciences › Medicine › Pharmacology › Musculoskeletal pain and rehabilitation
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Genetics › Connective tissue disorders research
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Cell Biology › Myofascial pain diagnosis and treatment
- Keywords
- Ehlers-Danlos syndrome, chronic pain, myofascial pain, central sensitization, complex regional pain syndrome, kinesiophobia, anxiety disorders, multidisciplinary pain management, pain self-management, cognitive behavioral therapy, graded exercise, connective tissue diseases
- MeSH terms
- Self-Management, Adult, Analgesics, Ehlers-Danlos Syndrome, Female, Humans, Pain Measurement, Cognitive Behavioral Therapy, Chronic Pain, Pain Management
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