Are patients with hypermobile Ehlers-Danlos syndrome or hypermobility spectrum disorder so different?

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License: CC BY 4.0
Serval ID
serval:BIB_C5E4147DA877
Type
Article: article from journal or magazin.
Collection
Publications
Institution
Title
Are patients with hypermobile Ehlers-Danlos syndrome or hypermobility spectrum disorder so different?
Journal
Rheumatology international
Author(s)
Aubry-Rozier B., Schwitzguebel A., Valerio F., Tanniger J., Paquier C., Berna C., Hügle T., Benaim C.
ISSN
1437-160X (Electronic)
ISSN-L
0172-8172
Publication state
Published
Issued date
10/2021
Peer-reviewed
Oui
Volume
41
Number
10
Pages
1785-1794
Language
english
Notes
Publication types: Journal Article
Publication Status: ppublish
Abstract
Diagnosing hypermobile Ehlers-Danlos syndrome (hEDS) remains challenging, despite new 2017 criteria. Patients not fulfilling these criteria are considered to have hypermobile spectrum disorder (HSD). Our first aim was to evaluate whether patients hEDS were more severely affected and had higher prevalence of extra-articular manifestations than HSD. Second aim was to compare their outcome after coordinated physical therapy. Patients fulfilling hEDS/HSD criteria were included in this real-life prospective cohort (November 2017/April 2019). They completed a 16-item Clinical Severity Score (CSS-16). We recorded bone involvement, neuropathic pain (DN4) and symptoms of mast cell disorders (MCAS) as extra-articular manifestations. After a standardized initial evaluation (T0), all patients were offered the same coordinated physical therapy, were followed-up at 6 months (T1) and at least 1 year later (T2), and were asked whether or not their condition had subjectively improved at T2. We included 97 patients (61 hEDS, 36 HSD). Median age was 40 (range 18-73); 92.7% were females. Three items from CSS-16 (pain, motricity problems, and bleeding) were significantly more severe with hEDS than HSD. Bone fragility, neuropathic pain and MCAS were equally prevalent. At T2 (20 months [range 18-26]) 54% of patients reported improvement (no difference between groups). On multivariable analysis, only family history of hypermobility predicted (favorable) outcome (p = 0.01). hEDS and HDS patients showed similar disease severity score except for pain, motricity problems and bleeding, and similar spectrum of extra-articular manifestations. Long-term improvement was observed in > 50% of patients in both groups. These results add weight to a clinical pragmatic proposition to consider hEDS/HSD as a single entity that requires the same treatments.
Keywords
Diagnosis-related groups, Ehlers–Danlos syndrome, Health care, Outcome assessment, Symptom assessment
Pubmed
Web of science
Open Access
Yes
Create date
03/09/2021 17:18
Last modification date
12/01/2022 7:13
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