Joint hypermobility should be assessed using the Beighton scale [Beighton et al., 1983]. Joint hypermobility depends on age, gender, family, and ethnic background. A score of 5/9 or greater
defines hypermobility. The total score is obtained by:
1forward flexion of the trunk with knees fully extended so that the palms of the hand rest flat* on the floor; one point
2hyperextension of the elbows beyond 10 degrees*; one point for each elbow
3hyperextension of the knees beyond 10 degrees*; one point for each knee
4passive apposition of the thumbs to the flexor aspect of the forearm*; one point for each hand
5passive dorsiflexion of the little fingers beyond 90 degrees*; one point for each hand
*Note: Picture may indicate a degree of hypermobility not required by the diagnostic criteria.
Brighton CriteriaAn important landmark was passed in July 2000 with the publication in the Journal of Rheumatology (2000; 27: 1777-1779) of the Brighton Diagnostic criteria for the Joint Hypermobility Syndrome (JHS).
Why is it important to have such a set of criteria for the JHS?
Hitherto, there has been no consensus view on how the JHS should be defined. Without generally agreed criteria for the diagnosis, it is very much a hit and miss affair and doctors confronted with an affected patient may come to very different conclusions regarding whether JHS is present. We know that that happens quite a lot. To take one example, doctors differ considerably in the number of hypermobile joints they require to be present before they accept the diagnosis on hypermobility syndrome. In a recent survey out of 319 consultant rheumatologists 185 required a Beighton score of 5 while 92 required 3, 3 required 1, while 35 preferred not commit themselves.
Why are the Brighton Criteria replacing the well tried Beighton Score?
The answer is that they are not. The Beighton scoring system has been used for over 30 years and have withstood the test of time. But as Professor Beighton has pointed out they were never designed for assisting in diagnosis in the clinical situation. They were in fact developed for epidemiological studies and for this they were invaluable.
They are not adequate for diagnosis for two reasons. Firstly, they only cover a sample of joints in the body, and reliance on them may lead to hypermobility being missed in other joints: those not covered in the Beighton 9-point score. Secondly, as we always knew, hypermobility syndrome means hypermobility + symptoms, and we now know there other features in JHS (changes in skin – increase skin stretchiness, thin scar formation, marfanoid body shape) so that a measurement of joint hypermobility alone is no longer enough. The new criteria include symptoms and these other features. The Beighton score remains an integral component. It is not being abandoned!
How reliable are the new Brighton Criteria?
The new criteria have been validated in adults but not yet in children below the age of 16 years. The process of validation requires the criteria to be "tested" among a group of JHS patients and also among a group of volunteers who have not got the syndrome. For criteria to declared valid they have to show that they are capable of picking up the condition in the majority who have it (sensitivity), but also of ruling out the condition in the majority of people who do not (specificity). In the case of the Brighton criteria the sensitivity and specificity were both very high (93% each).
How will they help?
Doctors and research workers can use the criteria in their work. If a person fulfils the criteria, then, for all intents and purposes they may be confidently considered to have the JHS. We therefore now have a reliable benchmark that we did not have before. It is anticipated that they will be widely used in future research. Indeed, since their publication they have already been incorporated into research protocols. For example, they will constitute an important linchpin in the gene-mapping project reported elsewhere in the website.
What do the Brighton Criteria consist of?
The actual criteria have been reproduced (as published) below.
Revised diagnostic criteria for the Joint Hypermobility Syndrome (JHS)
- A Beighton score of 4/9 or greater (either currently or historically)
- Arthralgia for longer than 3 months in 4 or more joints
- A Beighton score of 1, 2 or 3/9 (0, 1, 2 or 3 if aged 50+)
- Arthralgia (> 3 months) in one to three joints or back pain (> 3 months), spondylosis, spondylolysis/spondylolisthesis
- Dislocation/subluxation in more than one joint, or in one joint on more than one occasion
- Soft tissue rheumatism. > 3 lesions (e.g. epicondylitis, tenosynovitis, bursitis)
- Marfanoid habitus (tall, slim, span/height ratio >1.03, upper: lower segment ratio less than 0.89, arachnodactyly [positive Steinberg/wrist signs]
- Abnormal skin: striae, hyperextensibility, thin skin, papyraceous scarring
- Eye signs: drooping eyelids or myopia or antimongoloid slant
- Varicose veins or hernia or uterine/rectal prolapse.
The JHS is diagnosed in the presence two major criteria, or one major and two minor criteria, or four minor criteria. Two minor criteria will suffice where there is an unequivocally affected first-degree relative.
JHS is excluded by presence of Marfan or Ehlers-Danlos syndromes (other than the EDS Hypermobility type (formerly EDS III) as defined by the Ghent 1996 (8) and the Villefranche 1998 (9) criteria respectively). Criteria Major 1 and Minor 1 are mutually exclusive as are Major 2 and Minor 2.
Professor Rodney Grahame
About Joint Hypermobility Syndrome and EDSJoint Hypermobility Syndrome
is increasingly considered to be Hypermobility Type EDS
. Please see the following for more about this probable misdiagnosis:
"The Lack of Clinical Distinction Between the Hypermobility Type of Ehlers–Danlos Syndrome and the Joint Hypermobility Syndrome (a.k.a. Hypermobility Syndrome)" [Brad T. Tinkle, Howard A. Bird, Rodney Grahame, Mark Lavallee, Howard P. Levy, and David Sillence] available in PDF here
Dr. Rodney Grahame's presentation "EDS—A Rheumatologist's Perspective Over Four Decades" at the conference in 2011 in which he talks about the misunderstanding that benign hypermobility syndrome and EDS are separate, and that EDS is probably the most under-diagnosed disorder in the world, available from here
Dr. Heidi Collins, "Ehlers-Danlos Syndrome/Joint Hypermobility Syndrome: Are We Really Zebras, or Just Horses of Different Colors?" found here
"Dermal Ultrastructure in Low Beighton Score Members of 17 Families with Hypermobile-Type Ehlers-Danlos Syndrome" [Trinh Hermanns-Lê, Marie-Annick Reginster, Claudine Piérard-Franchimont, Philippe Delvenne, Gérald E. Piérard, and Daniel Manicourt] available from http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3471064
, stating "...we support the concept that BJHS represents a mild variant of EDSH."