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Review
. 2022 Mar 4;4(2):100189.
doi: 10.1016/j.arrct.2022.100189. eCollection 2022 Jun.

Exercise and Rehabilitation in People With Ehlers-Danlos Syndrome: A Systematic Review

Stephanie Buryk-Iggers  1   2 , Nimish Mittal  1   2   3 , Daniel Santa Mina  1   2   4 , Scott C Adams  1   5   6 , Marina Englesakis  7 , Maxim Rachinsky  2   4 , Laura Lopez-Hernandez  2 , Laura Hussey  2 , Laura McGillis  2 , Lianne McLean  2 , Camille Laflamme  2   3 , Dmitry Rozenberg  3   8 , Hance Clarke  2   3   4
Affiliations
Review

Exercise and Rehabilitation in People With Ehlers-Danlos Syndrome: A Systematic Review

Stephanie Buryk-Iggers et al. Arch Rehabil Res Clin Transl. .

Abstract

Objective: To conduct a systematic review examining the effect of exercise and rehabilitation in people with Ehlers-Danlos syndrome (EDS).

Data sources: The following databases were systematically searched: MEDLINE, MEDLINE In-Process/ePubs, Embase, Cochrane Central Register of Controlled Trials, PsycINFO, and Cumulative Index to Nursing and Allied Health. The final time point captured by the search is November 27, 2020.

Study selection: Eligible study designs included case-control, case-series, prospective cohort, retrospective cohort, and intervention studies of structured exercise or rehabilitation interventions. Eligible populations included adults (18 years or older) with EDS (all subtypes) and hypermobility spectrum disorders. The search was restricted to articles published in English.

Data extraction: Data were extracted by 2 independent reviewers. Risk of bias was assessed using the Physiotherapy Evidence Database (PEDro) scale for randomized controlled trials (RCTs) and Risk Of Bias In Nonrandomized Studies of Interventions (ROBINS-I) for non-RCTs. Reporting quality of RCTs was assessed using the Consolidated Standards for Reporting of Trials statement with the harms extension. Reporting was guided by the Preferred Reporting Items for Systematic Reviews and Meta-Analyses checklist.

Data synthesis: The search yielded 10 eligible studies including 330 participants. The study designs included 5 RCTs, 1 cohort, 2 single-arm interventions, 1 retrospective, and 1 feasibility study. All studies showed some improvement in a physical and/or psychological outcome after the intervention period. One adverse event (nonserious) potentially related to the intervention was reported. Of the 5 RCTs, 2 were rated as high quality with low risk of bias using PEDro, and the majority of non-RCTs were rated as critical risk of bias by ROBINS-I.

Conclusions: The results suggest that exercise and rehabilitation may be beneficial for various physical and psychological outcomes. Adequately powered and rigorous RCTs of exercise and rehabilitation interventions for people with EDS are needed.

Keywords: 6MWT, 6-minute walk test; AIMS-2, Arthritis Impact Measurement Scales-2; CONSORT, Consolidated Standards for Reporting of Trials; EDS, Ehlers-Danlos syndrome; Ehlers-Danlos Syndrome; Exercise; HADS, Hospital and Anxiety Depression Scale; HSD, hypermobility spectrum disorders; Joint instability; PEDro, Physiotherapy Evidence Database; PRISMA, Preferred Reporting Items for Systematic Reviews and Meta-Analyses; QOL, quality of life; RCT, randomized control trial; ROBINS-I, Risk Of Bias In Nonrandomized Studies of Interventions; Rehabilitation; SF-36, Short Form-36; VAS, visual analog scale; hEDS, hypermobile EDS.

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Figures

Fig 1
Fig 1
PRISMA diagram summarizing evidence search and selection (inclusive of secondary search and thus, 710 studies up to November 27, 2020).
See this image and copyright information in PMC

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