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Published in: Clinical Rheumatology 3/2021

01-03-2021 | Ehlers-Danlos Syndrome | Original Article

The effectiveness of conservative interventions for the management of syndromic hypermobility: a systematic literature review

Authors: Shea Palmer, Indi Davey, Laura Oliver, Amara Preece, Laura Sowerby, Sophie House

Published in: Clinical Rheumatology | Issue 3/2021

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Abstract

Introduction

‘Syndromic hypermobility’ encompasses heritable connective tissue disorders such as hypermobile Ehlers-Danlos syndrome and hypermobility spectrum disorders which are characterised by excessive joint range of motion and pain. Conservative interventions such as exercise are the cornerstone of management, yet their effectiveness is unclear.

Aim

To systematically appraise the effectiveness of conservative management for people with syndromic hypermobility.

Method

A systematic online database search was conducted (AMED, BND, CINAHL Plus, MEDLINE, PEDro, PsychINFO and SportDiscus). Potential articles were assessed for eligibility by two researchers against the following criteria: adults and children with a hEDS/HSD diagnosis (or equivalent diagnosis using specific criteria); non-pharmacological or non-surgical interventions; outcomes related to pain, physical function, psychological well-being or quality of life. Controlled trials and cohort studies were included. Critical Appraisal Skills Programme checklists were used to assess methodological quality.

Results

Eleven studies were included, comprising eight controlled trials and three cohort studies. All studies investigated interventions that had exercise as the primary component. Three small controlled studies demonstrated superior effects of conservative management relative to a control group. However, those studies only focused on a single area of the body, only recruited women, and had no long-term follow-up. All studies reported improvements in a wide range of outcomes over time.

Conclusion

Controlled trial evidence for the superiority of conservative management over comparators is weak. There is some evidence that people improve over time. Robust randomised controlled trial research of the long-term effectiveness of ‘whole-body’ (rather than individual joints or body areas) conservative management is required.
Key Points
• Conservative management is the cornerstone of management of syndromic hypermobility.
• The review found that evidence for the effectiveness of conservative management relative to no treatment or other conservative comparators was weak.
• However, there was consistent evidence for effectiveness from pre- to post-treatment.
• Further robust randomised controlled trial evidence is required.
Literature
2.
go back to reference Malfait F, Francomano C, Byers P, Belmont J, Berglund B, Black J, Bloom L, Bowen JM, Brady AF, Burrows NP, Castori M, Cohen H, Colombi M, Demirdas S, De Backer J, De Paepe A, Fournel-Gigleux S, Frank M, Ghali N, Giunta C, Grahame R, Hakim A, Jeunemaitre X, Johnson D, Juul-Kristensen B, Kapferer-Seebacher I, Kazkaz H, Kosho T, Lavallee ME, Levy H, Mendoza-Londono R, Pepin M, Pope FM, Reinstein E, Robert L, Rohrbach M, Sanders L, Sobey GJ, Van Damme T, Vandersteen A, van Mourik C, Voermans N, Wheeldon N, Zschocke J, Tinkle B (2017) The 2017 international classification of the Ehlers-Danlos syndromes. AmJ Med Genet C: Semin Med Genet 175(1):8–26. https://doi.org/10.1002/ajmg.c.31552CrossRef Malfait F, Francomano C, Byers P, Belmont J, Berglund B, Black J, Bloom L, Bowen JM, Brady AF, Burrows NP, Castori M, Cohen H, Colombi M, Demirdas S, De Backer J, De Paepe A, Fournel-Gigleux S, Frank M, Ghali N, Giunta C, Grahame R, Hakim A, Jeunemaitre X, Johnson D, Juul-Kristensen B, Kapferer-Seebacher I, Kazkaz H, Kosho T, Lavallee ME, Levy H, Mendoza-Londono R, Pepin M, Pope FM, Reinstein E, Robert L, Rohrbach M, Sanders L, Sobey GJ, Van Damme T, Vandersteen A, van Mourik C, Voermans N, Wheeldon N, Zschocke J, Tinkle B (2017) The 2017 international classification of the Ehlers-Danlos syndromes. AmJ Med Genet C: Semin Med Genet 175(1):8–26. https://​doi.​org/​10.​1002/​ajmg.​c.​31552CrossRef
4.
go back to reference Grahame R, Bird HA, Child A (2000) The revised (Brighton 1998) criteria for the diagnosis of benign joint hypermobility syndrome (BJHS). J Rheumatol 27(7):1777–1779PubMed Grahame R, Bird HA, Child A (2000) The revised (Brighton 1998) criteria for the diagnosis of benign joint hypermobility syndrome (BJHS). J Rheumatol 27(7):1777–1779PubMed
6.
go back to reference Tinkle B, Castori M, Berglund B, Cohen H, Grahame R, Kazkaz H, Levy H (2017) Hypermobile Ehlers-Danlos syndrome (a.k.a. Ehlers-Danlos syndrome type III and Ehlers-Danlos syndrome hypermobility type): clinical description and natural history. Am J Med Genet Part C Semin Med Genet 175C:48–69. https://doi.org/10.1002/ajmg.c.31538CrossRef Tinkle B, Castori M, Berglund B, Cohen H, Grahame R, Kazkaz H, Levy H (2017) Hypermobile Ehlers-Danlos syndrome (a.k.a. Ehlers-Danlos syndrome type III and Ehlers-Danlos syndrome hypermobility type): clinical description and natural history. Am J Med Genet Part C Semin Med Genet 175C:48–69. https://​doi.​org/​10.​1002/​ajmg.​c.​31538CrossRef
17.
go back to reference Sulli A, Talarico R, Scire CA, Avcin T, Castori M, Ferraris A, Frank C, Grunert J, Paolino S, Bombardieri S, Schneider M, Smith V, Cutolo M, Mosca M, Malfait F (2018) Ehlers-Danlos syndromes: state of the art on clinical practice guidelines. RMD Open 4(S1):e000790. https://doi.org/10.1136/2Frmdopen-2018-000790 Sulli A, Talarico R, Scire CA, Avcin T, Castori M, Ferraris A, Frank C, Grunert J, Paolino S, Bombardieri S, Schneider M, Smith V, Cutolo M, Mosca M, Malfait F (2018) Ehlers-Danlos syndromes: state of the art on clinical practice guidelines. RMD Open 4(S1):e000790. https://​doi.​org/​10.​1136/​2Frmdopen-2018-000790
27.
go back to reference Bale P, Easton V, Bacon H, Jerman E, Watts L, Barton G, Clark A, Armon K, MacGregor A (2019) The effectiveness of a multidisciplinary intervention strategy for the treatment of symptomatic joint hypermobility in childhood: a randomised, single centre parallel group trial (the Bendy study). Pediatr Rheumatol Online J 17(2):1–10. https://doi.org/10.1186/s12969-018-0298-xCrossRef Bale P, Easton V, Bacon H, Jerman E, Watts L, Barton G, Clark A, Armon K, MacGregor A (2019) The effectiveness of a multidisciplinary intervention strategy for the treatment of symptomatic joint hypermobility in childhood: a randomised, single centre parallel group trial (the Bendy study). Pediatr Rheumatol Online J 17(2):1–10. https://​doi.​org/​10.​1186/​s12969-018-0298-xCrossRef
28.
go back to reference Bathen T, Hangmann AB, Hoff M, Anderson LO, Rand-Hendriksen S (2013) Multidisciplinary treatment of disability in Ehlers-Danlos syndrome hypermobility type/hypermobility syndrome: a pilot study using a combination of physical and cognitive-behavioral therapy on 12 women. Am J Med Genet A 161A(12):3005–3011. https://doi.org/10.1002/ajmg.a.36060CrossRefPubMed Bathen T, Hangmann AB, Hoff M, Anderson LO, Rand-Hendriksen S (2013) Multidisciplinary treatment of disability in Ehlers-Danlos syndrome hypermobility type/hypermobility syndrome: a pilot study using a combination of physical and cognitive-behavioral therapy on 12 women. Am J Med Genet A 161A(12):3005–3011. https://​doi.​org/​10.​1002/​ajmg.​a.​36060CrossRefPubMed
35.
Metadata
Title
The effectiveness of conservative interventions for the management of syndromic hypermobility: a systematic literature review
Authors
Shea Palmer
Indi Davey
Laura Oliver
Amara Preece
Laura Sowerby
Sophie House
Publication date
01-03-2021
Publisher
Springer International Publishing
Published in
Clinical Rheumatology / Issue 3/2021
Print ISSN: 0770-3198
Electronic ISSN: 1434-9949
DOI
https://doi.org/10.1007/s10067-020-05284-0

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