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Review
. 2015 Aug 20:8:591-601.
doi: 10.2147/JPR.S64251. eCollection 2015.

Chronic pain in hypermobility syndrome and Ehlers-Danlos syndrome (hypermobility type): it is a challenge

Mark C Scheper  1 , Janneke E de Vries  2 , Jeanine Verbunt  3 , Raoul Hh Engelbert  1
Affiliations
Review

Chronic pain in hypermobility syndrome and Ehlers-Danlos syndrome (hypermobility type): it is a challenge

Mark C Scheper et al. J Pain Res. .

Abstract

Generalized joint hypermobility (GJH) is highly prevalent among patients diagnosed with chronic pain. When GJH is accompanied by pain in ≥4 joints over a period ≥3 months in the absence of other conditions that cause chronic pain, the hypermobility syndrome (HMS) may be diagnosed. In addition, GJH is also a clinical sign that is frequently present in hereditary diseases of the connective tissue, such as the Marfan syndrome, osteogenesis imperfecta, and the Ehlers-Danlos syndrome. However, within the Ehlers-Danlos spectrum, a similar subcategory of patients having similar clinical features as HMS but lacking a specific genetic profile was identified: Ehlers-Danlos syndrome hypermobility type (EDS-HT). Researchers and clinicians have struggled for decades with the highly diverse clinical presentation within the HMS and EDS-HT phenotypes (Challenge 1) and the lack of understanding of the pathological mechanisms that underlie the development of pain and its persistence (Challenge 2). In addition, within the HMS/EDS-HT phenotype, there is a high prevalence of psychosocial factors, which again presents a difficult issue that needs to be addressed (Challenge 3). Despite recent scientific advances, many obstacles for clinical care and research still remain. To gain further insight into the phenotype of HMS/EDS-HT and its mechanisms, clearer descriptions of these populations should be made available. Future research and clinical care should revise and create consensus on the diagnostic criteria for HMS/EDS-HT (Solution 1), account for clinical heterogeneity by the classification of subtypes within the HMS/EDS-HT spectrum (Solution 2), and create a clinical core set (Solution 3).

Keywords: Ehlers-Danlos hypermobility type; chronic musculoskeletal pain; generalized joint hypermobility; hypermobility syndrome.

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Figures

Figure 1
Figure 1
Clinical heterogeneity. Abbreviation: GJH, generalized joint hypermobility.
Figure 2
Figure 2
Potential pathways of chronic pain in GJH-related disorders. Abbreviation: GJH, generalized joint hypermobility.
See this image and copyright information in PMC

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References

    1. Greenwood NL, Duffell LD, Alexander CM, McGregor AH. Electromyographic activity of pelvic and lower limb muscles during postural tasks in people with benign joint hypermobility syndrome and non hypermobile people. A pilot study. Man Ther. 2011;16(6):623–628. - PMC - PubMed
    1. Kirk JA, Ansell BM, Bywaters EG. The hypermobility syndrome. Musculoskeletal complaints associated with generalized joint hypermobility. Ann Rheum Dis. 1967;26(5):419–425. - PMC - PubMed
    1. Scheper MC, Engelbert RH, Rameckers EA, Verbunt J, Remvig L, Juul-Kristensen B. Children with generalised joint hypermobility and musculoskeletal complaints: state of the art on diagnostics, clinical characteristics, and treatment. Biomed Res Int. 2013;2013:121054. - PMC - PubMed
    1. Grahame R. Hypermobility: an important but often neglected area within rheumatology. Nat Clin Pract Rheumatol. 2008;4(10):522–524. - PubMed
    1. Grahame R, Bird HA, Child A. The revised (Brighton 1998) criteria for the diagnosis of benign joint hypermobility syndrome (BJHS) J Rheumatol. 2000;27(7):1777–1779. - PubMed