Easily Missed?

Joint hypermobility syndrome

BMJ 2011; 342 doi: http://dx.doi.org/10.1136/bmj.c7167 (Published 20 January 2011)
Cite this as: BMJ 2011;342:c7167

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  1. Juliette Ross, GP principal1,
  2. Rodney Grahame, consultant rheumatologist2, honorary professor 3, affiliate professor of pathology4
  1. 1Wembley Park Drive Medical Centre, Wembley, UK
  2. 2Hypermobility Clinic, University College Hospital, London NW1 2PQ, UK
  3. 3Department of Medicine, University College London, London
  4. 4School of Medicine, University of Washington, Seattle, WA, USA
  1. Correspondence to: R Grahame rodney.grahame{at}uclh.nhs.uk

Joint hypermobility syndrome (JHS), previously known as benign joint hypermobility syndrome (BJHS), is a heritable disorder of connective tissue that comprises symptomatic hypermobility predisposing to arthralgia, soft tissue injury, and joint instability.1 It is indistinguishable from the hypermobility type of Ehlers-Danlos syndrome.2 Complications may include autonomic dysfunction, proprioceptive impairment, premature osteoarthritis, intestinal dysmotility, and laxity in other tissues causing hernias or uterine or rectal prolapse. Symptoms are often minimal or mild, but 168 out of 700 patients with joint hypermobility syndrome (24%) attending the UCH Hypermobility Clinic already had an established chronic pain syndrome at the time of their first outpatient attendance. These patients were experiencing serious pain, disability, and impairment of the quality of life, some patients becoming chairbound or even bedbound.3

Case scenario

A 30 year old project manager, who is new to your general practice, presents with right anterior knee pain after slipping and landing on his knee three months ago. Imaging shows no abnormality, but he describes a long history of recurrent shoulder subluxation, and many soft tissue problems and joint pains, often after similarly trivial trauma, and he states that imaging and blood tests “for arthritis” have always been normal. You note that he has no signs of inflammation but that he is hypermobile according to the Beighton score (see box 1), and looking up the Brighton criteria, which includes and extends the older Beighton score (see box 2), you mention he fulfils the criteria for joint hypermobility syndrome, and he expresses relief there is an explanation for his symptoms.

Box 1 Nine-point Beighton score for joint hypermobility syndrome4

One point is gained for each side of the body for the first four manoeuvres listed below, such that the hypermobility score is a maximum of 9 if all are positive.

  • Passive dorsiflexion of the fifth metacarpophalangeal joint to ≥90° (1 point for left; …

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