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Cited by 61Connective tissue disorders researchMusculoskeletal pain and rehabilitationOral and gingival health research

Time to take hypermobility seriously (in adults and children)

Grahame, R.·2001·Rheumatology
DOI 10.1093/rheumatology/40.5.485
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Abstract

A third of a century has elapsed since the hypermobility syndrome (HMS) appeared on the rheumatological horizon [1]. From the outset it was perceived more as a curiosity than as an entity that could have significant (let alone serious) import. In both children and adults it aroused more wonder at the seemingly bizarre contortionist manoeuvres patients could perform (to the misplaced amusement of medical students and their teachers) than a quest for appreciating and alleviating the adverse aspects of the condition. There is now abundant evidence from papers published in peer‐reviewed journals in many countries to demonstrate the serious impact that hypermobility can have on peoples' lives [2]. There is no longer (if there ever was) any justification for regarding hypermobility as merely a circus act [3]. The original description of Kirk et al. [1] defined the HMS as the occurrence of ‘musculoskeletal symptoms in the presence of generalised joint laxity in otherwise normal subjects’. It was diagnosis by exclusion, the phrase ‘otherwise normal’ implying the absence of other identifiable rheumatic disease. Yet much has been learnt since to change our concept of the condition. It has been established from a number of studies [4, 5], first that hypermobility is more often pauciarticular than polyarticular and, secondly that it does not have to be generalized to cause symptoms [6]. Even a single hypermobile joint may suffer any or all of the consequences of laxity, including a tendency to dislocate, develop traumatic synovitis or premature osteoarthritis, or it may just hurt for no visibly obvious reason [2]. Thirdly, the HMS, as seen from the clinic perspective, far from occurring in ‘otherwise normal subjects’, has all the hallmarks of a forme fruste of a heritable disorder of connective tissue (HDCT). That is to say, it is imbued with features that overlap with its more serious cousins—the Marfan and Ehlers–Danlos (EDS) syndromes and osteogenesis imperfecta—though in general its features are milder and lesser in degree [7]. As life expectancy is not reduced, the use of the term ‘benign joint hypermobility syndrome’ (BJHS) has largely replaced the earlier HMS [8]. The British Society for Rheumatology (BSR) Special Interest Group on the HDCTs has recently published a revised set of validated diagnostic criteria for the BJHS [9], which many authorities now believe to be identical with the hypermobility type of the EDS (formerly EDS type III [10]). The BJHS represents a complex mix of acute, recurrent or recalcitrant, widespread soft tissue lesions of traumatic origin, recurrent joint subluxations and/or dislocations, often commencing in childhood or adolescence and continuing into adult life. This painful existence may be compounded by as yet undetermined neurophysiological influences, possibly including nociceptive enhancement. Clues to this possibility are provided by the discovery of two seemingly unrelated, though tantalizing, observations, namely that patients with BJHS/EDS have diminished joint proprioceptive acuity [11] and are less responsive than normal to the local anaesthetic effects of lignocaine [12]. Failure to receive effective intervention not infrequently results in a complicating chronic pain syndrome [13], fibromyalgia [14] and depression [15]. Later in life, premature osteoarthritis may add to the burden of morbidity [2].

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Crossref · 10.1093/rheumatology/40.5.485

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  • Having joint hypermobility implies increased flexibility but also a propensity for trauma and pain because the tissues are more fragile.

    “To have JH implies increased flexibility but also a propensity for trauma and pain since the tissues are more fragile.”

    From Autism, joint hypermobility-related disorders and pain (2018) · cites this paper as [18]

  • Even a single hypermobile joint may suffer any or all of the consequences of laxity, including a tendency to dislocate, develop traumatic synovitis or premature osteoarthritis, or it may just hurt for no visibly obvious reason.

    “According to Grahame [(18) p. 485] “Even a single hypermobile joint may suffer any or all of the consequences of laxity, including a tendency to dislocate, develop traumatic synovitis or premature osteoarthritis, or it may just hurt for no visibly obvious reason.””

    From Autism, joint hypermobility-related disorders and pain (2018) · cites this paper as [18]

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Authors
Grahame, R.
Affiliations
University College Hospital (GB)
Corresponding author
Grahame, R.
Venue
Rheumatology
Publisher
Oxford University Press (OUP)
Published
2001-05-01
Volume
40
Issue
5
Pages
485-487
ISSN
  • 0263-7103 (ISSN-L)
  • 1460-2172
DOI
10.1093/rheumatology/40.5.485
OpenAlex
W2089886881
Cited by
61
References
25
Citations by year
2024: 1 · 2022: 1 · 2021: 1 · 2020: 3 · 2018: 2 · 2017: 4 · 2016: 1 · 2015: 7 · 2014: 2 · 2013: 3 · 2012: 5
FWCI
1.27
Type
journal article
Language
en
Open access
closed
Locations
  • British journal of rheumatology (publishedVersion)
  • PubMed (publishedVersion)
Topics
  • Life Sciences › Biochemistry, Genetics and Molecular Biology › Genetics › Connective tissue disorders research
  • Health Sciences › Medicine › Pharmacology › Musculoskeletal pain and rehabilitation
  • Health Sciences › Health Professions › Pharmacy › Oral and gingival health research
Keywords
benign joint hypermobility syndrome, hypermobility syndrome, hereditary connective tissue disorders, Ehlers-Danlos syndrome, Marfan syndrome, osteogenesis imperfecta, joint laxity, diagnosis, diagnostic criteria, musculoskeletal symptoms, dislocations, premature osteoarthritis, children, adults, quality of life
MeSH terms
Adult, Child, Humans, Joint Instability, Rheumatology

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