The Brighton Score - The New Diagnostic Criteria for HMS

An 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 Benign Joint Hypermobility Syndrome (BJHS).

WHY IS IT IMPORTANT TO HAVE SUCH A SET OF CRITERIA FOR THE BJHS?

Hitherto, there has been no consensus view on how the BJHS 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 BJHS 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 BJHS (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 BJHS 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 BJHS. 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 BENIGN JOINT HYPERMOBILITY SYNDROME (BJHS)

Major Criteria

• A Beighton score of 4/9 or greater (either currently or historically)
• Arthralgia for longer than 3 months in 4 or more joints

Minor Criteria

• 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, arachnodactily [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 BJHS 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.

BJHS 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

UCL Hospitals, LONDON

Journal of Rheumatology (2000; 27: 1777-1779) Brighton Diagnostic Criteria for the Benign Joint Hypermobility Syndrome (BJHS)