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

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

Type
article
Institution
UNIL/CHUV/Unisanté + institutions partenaires
Périodique
Rheumatology International  
Auteur(s)
Aubry-Rozier, B.
Auteure/Auteur
Schwitzguebel, A.
Auteure/Auteur
Valerio, F.
Auteure/Auteur
Tanniger, J.
Auteure/Auteur
Paquier, C.
Auteure/Auteur
Berna, C.
Auteure/Auteur
HĂĽgle, T.
Auteure/Auteur
Benaim, C.
Auteure/Auteur
Liens vers les personnes
Benaim, Charles  
Berna Renella, Chantal  
Paquier, CĂ©line  
HĂĽgle, Thomas  
Liens vers les unités
AnesthĂ©siologie  
Rhumatologie  
MĂ©decine physique et rĂ©habilitation  
ISSN
1437-160X
Statut éditorial
Publié
Date de publication
2021-10
Volume
41
Numéro
10
Première page
1785
Dernière page/numéro d’article
1794
Peer-reviewed
Oui
Langue
anglais
Notes
Publication types: Journal Article
Publication Status: ppublish
Résumé
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.
Sujets

Diagnosis-related gro...

Ehlers–Danlos syndrom...

Health care

Outcome assessment

Symptom assessment

PID Serval
serval:BIB_C5E4147DA877
DOI
10.1007/s00296-021-04968-3
PMID
34398260
WOS
000685380900001
Permalien
https://iris.unil.ch/handle/iris/185199
Open Access
Oui
Date de création
2021-09-03T16:18:51.940Z
Date de création dans IRIS
2025-05-21T01:20:54Z
Fichier(s)
En cours de chargement...
Vignette d'image
Nom

34398260_BIB_C5E4147DA877.pdf

Version du manuscrit

published

Licence

https://creativecommons.org/licenses/by/4.0

Taille

513.74 KB

Format

Adobe PDF

PID Serval

serval:BIB_C5E4147DA877.P001

URN

urn:nbn:ch:serval-BIB_C5E4147DA8772

Somme de contrĂ´le

(MD5):d4e45db92483784b30c3294b31d003d7

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