Quantitative measures of tissue mechanics to detect hypermobile Ehlers-Danlos syndrome and hypermobility syndrome disorders: a systematic review

Shea Palmer, Elise Denner, Matthew Riglar, Holly Scannell, Sarah Webb, Georgina Young

Research output: Contribution to journalReview articlepeer-review

6 Citations (Scopus)
17 Downloads (Pure)

Abstract

Hypermobile Ehlers-Danlos syndrome (hEDS) and hypermobility spectrum disorders (HSD) are heritable connective tissue disorders associated with pain, activity limitations and participation restrictions. A key feature is reported to be reduced stiffness and increased extensibility and elasticity of connective tissues. Yet diagnosis relies on assessment of joint range of motion, which may be influenced by other factors, and semi-quantitative assessment of forearm skin extensibility. The objective of this systematic review was to determine if quantitative measures of tissue mechanics can discriminate between hEDS/HSD and healthy tissues. Literature was identified via online databases (AMED, CINAHL+, EMBASE, MEDLINE and SportDiscus) and snowballing. Studies were included if participants had a confirmed diagnosis of hEDS/HSD (or equivalent diagnosis) using internationally recognised criteria, a healthy control group was used as a comparator, and objective measures of tissue stiffness, extensibility or elasticity of muscle, tendon, connective tissue or skin were reported. Included studies were critically appraised, followed by group discussion, consensus and narrative synthesis. Two hundred three potentially relevant studies were identified. Application of the inclusion criteria resulted in four studies being included. A range of quantitative approaches to studying tissue mechanics were used, including diagnostic ultrasound. Overall, three of the four studies found that at least one measure of tissue mechanics distinguished between people with hEDS/HSD and healthy controls. The studies were generally conducted and reported to high standards. Quantitative measures of tissue mechanics have the potential to contribute towards more objective diagnosis of hEDS/HSD. Further validation, particularly within diagnostic scenarios, is required.

Original languageEnglish
Pages (from-to)715-725
Number of pages11
JournalClinical Rheumatology
Volume39
Early online date18 Jan 2020
DOIs
Publication statusPublished - 1 Mar 2020
Externally publishedYes

Bibliographical note

Open Access This article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/.

Keywords

  • Connective tissue diseases
  • Ehlers-Danlos syndrome
  • Elasticity
  • Familial
  • Joint laxity

ASJC Scopus subject areas

  • Rheumatology

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