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Central sensitization as the mechanism underlying pain in joint hypermobility syndrome/Ehlers-Danlos syndrome, hypermobility type.
Eur J Pain 2016; 20(8):1319-25EJ

Abstract

BACKGROUND

Patients with joint hypermobility syndrome/Ehlers-Danlos syndrome, hypermobility type (JHS/EDS-HT) commonly suffer from pain. How this hereditary connective tissue disorder causes pain remains unclear although previous studies suggested it shares similar mechanisms with neuropathic pain and fibromyalgia.

METHODS

In this prospective study seeking information on the mechanisms underlying pain in patients with JHS/EDS-HT, we enrolled 27 consecutive patients with this connective tissue disorder. Patients underwent a detailed clinical examination, including the neuropathic pain questionnaire DN4 and the fibromyalgia rapid screening tool. As quantitative sensory testing methods, we included thermal-pain perceptive thresholds and the wind-up ratio and recorded a standard nerve conduction study to assess non-nociceptive fibres and laser-evoked potentials, assessing nociceptive fibres.

RESULTS

Clinical examination and diagnostic tests disclosed no somatosensory nervous system damage. Conversely, most patients suffered from widespread pain, the fibromyalgia rapid screening tool elicited positive findings, and quantitative sensory testing showed lowered cold and heat pain thresholds and an increased wind-up ratio.

CONCLUSIONS

While the lack of somatosensory nervous system damage is incompatible with neuropathic pain as the mechanism underlying pain in JHS/EDS-HT, the lowered cold and heat pain thresholds and increased wind-up ratio imply that pain in JHS/EDS-HT might arise through central sensitization. Hence, this connective tissue disorder and fibromyalgia share similar pain mechanisms. WHAT DOES THIS STUDY ADD?: In patients with JHS/EDS-HT, the persistent nociceptive input due to joint abnormalities probably triggers central sensitization in the dorsal horn neurons and causes widespread pain.

Authors+Show Affiliations

Department of Neurology and Psychiatry, Sapienza University, Rome, Italy.Physical Medicine and Rehabilitation Division, Policlinico Umberto I, Sapienza University, Rome, Italy.Division of Neurological Pain Research and Therapy, Universitätsklinikum Schleswig-Holstein, Kiel, Germany.Division of Medical Genetics, Department of Molecular Medicine, San Camillo-Forlanini Hospital, Sapienza University, Rome, Italy.Rheumatology Unit, Department of Internal Medicine and Medical Specialities, Sapienza University, Rome, Italy.Department of Neurology and Psychiatry, Sapienza University, Rome, Italy.Department of Neurology and Psychiatry, Sapienza University, Rome, Italy.Department of Neurology and Psychiatry, Sapienza University, Rome, Italy.Department of Neurology and Psychiatry, Sapienza University, Rome, Italy.Department of Neurology and Psychiatry, Sapienza University, Rome, Italy.Physical Medicine and Rehabilitation Division, Policlinico Umberto I, Sapienza University, Rome, Italy.

Pub Type(s)

Journal Article

Language

eng

PubMed ID

26919608

Citation

Di Stefano, G, et al. "Central Sensitization as the Mechanism Underlying Pain in Joint Hypermobility syndrome/Ehlers-Danlos Syndrome, Hypermobility Type." European Journal of Pain (London, England), vol. 20, no. 8, 2016, pp. 1319-25.
Di Stefano G, Celletti C, Baron R, et al. Central sensitization as the mechanism underlying pain in joint hypermobility syndrome/Ehlers-Danlos syndrome, hypermobility type. Eur J Pain. 2016;20(8):1319-25.
Di Stefano, G., Celletti, C., Baron, R., Castori, M., Di Franco, M., La Cesa, S., ... Camerota, F. (2016). Central sensitization as the mechanism underlying pain in joint hypermobility syndrome/Ehlers-Danlos syndrome, hypermobility type. European Journal of Pain (London, England), 20(8), pp. 1319-25. doi:10.1002/ejp.856.
Di Stefano G, et al. Central Sensitization as the Mechanism Underlying Pain in Joint Hypermobility syndrome/Ehlers-Danlos Syndrome, Hypermobility Type. Eur J Pain. 2016;20(8):1319-25. PubMed PMID: 26919608.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Central sensitization as the mechanism underlying pain in joint hypermobility syndrome/Ehlers-Danlos syndrome, hypermobility type. AU - Di Stefano,G, AU - Celletti,C, AU - Baron,R, AU - Castori,M, AU - Di Franco,M, AU - La Cesa,S, AU - Leone,C, AU - Pepe,A, AU - Cruccu,G, AU - Truini,A, AU - Camerota,F, Y1 - 2016/02/26/ PY - 2016/01/10/accepted PY - 2016/2/27/entrez PY - 2016/2/27/pubmed PY - 2018/1/11/medline SP - 1319 EP - 25 JF - European journal of pain (London, England) JO - Eur J Pain VL - 20 IS - 8 N2 - BACKGROUND: Patients with joint hypermobility syndrome/Ehlers-Danlos syndrome, hypermobility type (JHS/EDS-HT) commonly suffer from pain. How this hereditary connective tissue disorder causes pain remains unclear although previous studies suggested it shares similar mechanisms with neuropathic pain and fibromyalgia. METHODS: In this prospective study seeking information on the mechanisms underlying pain in patients with JHS/EDS-HT, we enrolled 27 consecutive patients with this connective tissue disorder. Patients underwent a detailed clinical examination, including the neuropathic pain questionnaire DN4 and the fibromyalgia rapid screening tool. As quantitative sensory testing methods, we included thermal-pain perceptive thresholds and the wind-up ratio and recorded a standard nerve conduction study to assess non-nociceptive fibres and laser-evoked potentials, assessing nociceptive fibres. RESULTS: Clinical examination and diagnostic tests disclosed no somatosensory nervous system damage. Conversely, most patients suffered from widespread pain, the fibromyalgia rapid screening tool elicited positive findings, and quantitative sensory testing showed lowered cold and heat pain thresholds and an increased wind-up ratio. CONCLUSIONS: While the lack of somatosensory nervous system damage is incompatible with neuropathic pain as the mechanism underlying pain in JHS/EDS-HT, the lowered cold and heat pain thresholds and increased wind-up ratio imply that pain in JHS/EDS-HT might arise through central sensitization. Hence, this connective tissue disorder and fibromyalgia share similar pain mechanisms. WHAT DOES THIS STUDY ADD?: In patients with JHS/EDS-HT, the persistent nociceptive input due to joint abnormalities probably triggers central sensitization in the dorsal horn neurons and causes widespread pain. SN - 1532-2149 UR - https://www.unboundmedicine.com/medline/citation/26919608/Central_sensitization_as_the_mechanism_underlying_pain_in_joint_hypermobility_syndrome/Ehlers_Danlos_syndrome_hypermobility_type_ L2 - https://doi.org/10.1002/ejp.856 DB - PRIME DP - Unbound Medicine ER -