Cited by 237Connective tissue disorders researchMusculoskeletal pain and rehabilitationPediatric Pain Management Techniques

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Pain in Ehlers-Danlos Syndrome Is Common, Severe, and Associated with Functional Impairment

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Voermans, Nicol C., Knoop, Hans, Bleijenberg, Gijs, van Engelen, Baziel G.·2010·Journal of Pain and Symptom Management

DOI 10.1016/j.jpainsymman.2009.12.026

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Abstract

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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Authors: Voermans, N. C., Knoop, H., Bleijenberg, G., & van Engelen, B. G.
Affiliations: Radboud University Medical Center (NL)
Corresponding author: Voermans, Nicol C.
ORCID: Voermans, Nicol C.
Knoop, Hans
Bleijenberg, Gijs
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)
DOI: 10.1016/j.jpainsymman.2009.12.026
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: www.elsevier.com/tdm/userlicense/1.0/ (tdm)
www.elsevier.com/legal/tdmrep-license (tdm)
www.elsevier.com/open-access/userlicense/1.0/ (vor, 3421 d)
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 › 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 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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Pain in Ehlers-Danlos Syndrome Is Common, Severe, and Associated with Functional Impairment

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