Abstract
Objective
To (1) establish the association of the most common reported symptoms on disability;
and (2) study the effectiveness of treatment on disability in patients with Ehlers-Danlos
syndrome–hypermobility type (EDS-HT)/hypermobility syndrome (HMS).
Data Sources
An electronic search (Medical Subject Headings and free-text terms) was conducted
in bibliographic databases CENTRAL/MEDLINE.
Study Selection
Comparative, cross-sectional, longitudinal cohort studies and (randomized) controlled
trials including patients with HMS/EDS-HT aged ≥17 years were considered for inclusion.
A class of symptoms was included when 5 publications were available. In regards to
treatment (physical, cognitive interventions), only (randomized) controlled trials
were considered. Surgical and medicinal interventions were excluded.
Data Extraction
Bias was assessed according to the methodological scoring tools of the Cochrane collaboration.
Z-score transformations were applied to classify the extent of disability in comparison
with healthy controls and to ensure comparability between studies.
Data Synthesis
Initially, the electronic search yielded 714 publications, and 21 articles remained
for analysis after selection. The following symptoms were included for meta-analysis:
pain (n=12), fatigue (n=6), and psychological distress (n=7). Pain (r=.64, P=.021), fatigue (r=.91, P=.011), and psychological distress (r=.86, P=.018) had a significant impact on disability. Regarding treatment, a significant
pain reduction was achieved by a variety of physical and cognitive approaches. Treatment
effectiveness on disability was not established.
Conclusions
Disability can affect patients with HMS/EDS-HT significantly and is highly correlated
with both physical and psychological factors. Although evidence is available that
physical and psychological treatment modalities can induce significant pain reduction,
the evidence regarding disability reduction is lacking.
Keywords
List of abbreviations:
EDS (Ehlers-Danlos syndrome), EDS-HT (Ehlers-Danlos syndrome–hypermobility type), GJH (generalized joint hypermobility), HDCT (hereditary diseases of connective tissue), HMS (hypermobility syndrome), ICF (International Classification of Functioning, Disability and Health), IQR (interquartile range), MeSH (Medical Subject Headings)To read this article in full you will need to make a payment
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Article info
Publication history
Published online: March 11, 2016
Footnotes
Supported by the Dutch Society for Scientific Research (NWO), the University of Applied Sciences Amsterdam, Education for Physical Therapy (grant no. 023.002.094).
Disclosures: none.
Identification
Copyright
© 2016 by the American Congress of Rehabilitation Medicine
