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

Scheper MC, de Vries JE, Verbunt J, Engelbert RH - J Pain Res (2015)

Bottom Line: 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).

View Article: PubMed Central - PubMed

Affiliation: School of Physiotherapy, Amsterdam University of Applied Sciences, Amsterdam, the Netherlands ; Department of Rehabilitation, Academic Medical Center, University of Amsterdam, Amsterdam, the Netherlands.

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).

No MeSH data available.


Related in: MedlinePlus

Clinical heterogeneity.Abbreviation: GJH, generalized joint hypermobility.
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f1-jpr-8-591: Clinical heterogeneity.Abbreviation: GJH, generalized joint hypermobility.

Mentions: From a diagnostic perspective, only one type of HMS/EDS-HT was historically distinguished.22 When considering the highly prevalent nature of GJH, and the (estimated) incidence rate of HMS/EDS-HT, not all subjects with GJH become patients. It has been acknowledged that one of the trademarks of HMS/EDS-HT is the clinical heterogeneity;23 however, this can be problematic for clinicians during diagnosis and treatment.4 The presence of clinical heterogeneity could imply the existence of subtypes within the spectrum of HMS/EDS-HT (Figure 1).24,25


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

Scheper MC, de Vries JE, Verbunt J, Engelbert RH - J Pain Res (2015)

Clinical heterogeneity.Abbreviation: GJH, generalized joint hypermobility.
© Copyright Policy
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4548768&req=5

f1-jpr-8-591: Clinical heterogeneity.Abbreviation: GJH, generalized joint hypermobility.
Mentions: From a diagnostic perspective, only one type of HMS/EDS-HT was historically distinguished.22 When considering the highly prevalent nature of GJH, and the (estimated) incidence rate of HMS/EDS-HT, not all subjects with GJH become patients. It has been acknowledged that one of the trademarks of HMS/EDS-HT is the clinical heterogeneity;23 however, this can be problematic for clinicians during diagnosis and treatment.4 The presence of clinical heterogeneity could imply the existence of subtypes within the spectrum of HMS/EDS-HT (Figure 1).24,25

Bottom Line: 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).

View Article: PubMed Central - PubMed

Affiliation: School of Physiotherapy, Amsterdam University of Applied Sciences, Amsterdam, the Netherlands ; Department of Rehabilitation, Academic Medical Center, University of Amsterdam, Amsterdam, the Netherlands.

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).

No MeSH data available.


Related in: MedlinePlus