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Kathryn Bailey, Geoff Debelle, Comment on: The multisystemic nature and natural history of joint hypermobility syndrome and Ehlers-Danlos syndrome in children, Rheumatology, Volume 57, Issue 12, December 2018, Pages 2248–2250, https://doi.org/10.1093/rheumatology/key251
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Sir, Representatives of The Royal College of Paediatrics and Child Health (RCPCH) and The British Society of Paediatric and Adolescent Rheumatology (BSPAR) express concerns regarding Professor Grahame’s personal writing [1] and believe this editorial raises serious questions relating to the healthcare and safety of children. In particular, we have concerns that non-specific systemic symptoms and functional difficulties are diagnosed as hypermobile Ehlers Danlos syndrome (hEDS), limiting the effectiveness of rehabilitation and, more worryingly, suggesting signs of physical abuse can be attributed to hEDS.
The paper by Dr Scheper et al. [2] that this editorial accompanies should be commended and the British Society of Rheumatology/RCPCH and wider community would encourage this group to re-examine their data using the current hEDS criteria (utilizing Beighton score 6) [3]. This will prove a valuable addition to other studies evaluating the postulated association between global joint laxity in the growing child and morbidity, in and above that already well described and published in childhood chronic musculoskeletal pain (with no hypermobility) [4–6]. The study describes a relatively small cohort of children within a wide age bracket, utilizing hEDS criteria that have now been updated and modified to reflect concerns relating to over-interpretation of non-specific symptoms and clinical features. Other larger population studies show that a Beighton score 4 is not useful in assessing the developing skeleton [7, 8] and that basic normative joint ranges in children and young people need to be evaluated and published. The 2017 International Classification of the EDS takes this into account, and importantly does not include systemic manifestations as part of the diagnostic criteria for hEDS [8]. As the authors are aware, there is no evidence to date (genetic, tissue or otherwise) of collagen abnormalities in this cohort [9].
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