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

Stephanie Buryk-Iggers; Nimish Mittal; Daniel Santa Mina; Scott C Adams; Marina Englesakis; Maxim Rachinsky; Laura Lopez-Hernandez; Laura Hussey; Laura McGillis; Lianne McLean; Camille Laflamme; Dmitry Rozenberg; Hance Clarke

doi:10.1016/j.arrct.2022.100189

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

Arch Rehabil Res Clin Transl. 2022 Mar 4;4(2):100189. doi: 10.1016/j.arrct.2022.100189. eCollection 2022 Jun.

Authors

Stephanie Buryk-Iggers^{1

2}, Nimish Mittal^{1

2

3}, Daniel Santa Mina^{1

2

4}, Scott C Adams^{1

5

6}, Marina Englesakis⁷, Maxim Rachinsky^{2

4}, Laura Lopez-Hernandez², Laura Hussey², Laura McGillis², Lianne McLean², Camille Laflamme^{2

3}, Dmitry Rozenberg^{3

8}, Hance Clarke^{2

3

4}

Affiliations

¹ Department of Kinesiology and Physical Education, University of Toronto, Toronto, Ontario, Canada.
² GoodHope Ehlers Danlos Syndrome Clinic, Toronto General Hospital, Toronto, Ontario, Canada.
³ Faculty of Medicine, Division of Physical Medicine and Rehabilitation, University of Toronto, Toronto, Ontario, Canada.
⁴ Department of Anaesthesia and Pain Management, University Health Network, Toronto, Ontario, Canada.
⁵ Department of Cardiology, Toronto General Hospital Research Institute, Toronto, Ontario, Canada.
⁶ Ted Rogers Cardiotoxicity Prevention Program, Peter Munk Cardiac Centre, Toronto, Ontario, Canada.
⁷ Library and Information Services Toronto General Hospital, Toronto, Ontario, Canada.
⁸ Department of Medicine, Respirology, Toronto General Hospital Research Institute, Toronto, Ontario, Canada.

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.

Publication types

Review