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The Brighton Criteria for JHS

Posted By Donna Wicks, March 16, 2017

This page has been updated in light of the 2017 International Criteria for Ehlers-Danlos syndrome.

Although the terms Hypermobility Spectrum Disorder (HSD) and Hypermobile Ehlers-Danlos syndrome (hEDS) now apply, and the term Joint Hypermobility Syndrome (JHS) has been dropped, it is important that information on JHS is still presented given its historical context both as a medical diagnosis and with regard to previous research.

Many people have a diagnosis of JHS. In most cases, if the term JHS alone was used to describe the diagnosis then it is most likely that the term HSD would now apply.

For those individuals that were given a diagnosis of JHS/EDS-HT (i.e. the doctor felt the concerns were more in keeping with the hypermobile variant of EDS) it would be anticipated that these individuals have hEDS given the 2017 criteria aims to identify both the more complex end of JHS and better define Hypermobile EDS.

There will be some individuals with JHS where the term EDS-HT / EDS III has not been used but who are now concerned that they may have hEDS based on the 2017 Criteria. The HMSA advises that they seek their doctors’ opinion in the first instance.

The Diagnostic Criteria for Joint Hypermobility Syndrome (JHS)

Why was it important to have such a set of criteria for the JHS?

Prior to the late 1990s there was no consensus view on how JHS should be defined. Without generally agreed criteria for the diagnosis, it was very much a hit and miss affair and doctors confronted with an affected patient may come to very different conclusions as to whether JHS was present or not. Doctors therefore used the criteria to confidently make the diagnosis of JHS. It was also widely used in research, and has been an important way of comparing the associations with other signs and symptoms found in different patient groups; for example the studies on cardiovascular autonomic dysfunction and bowel problems. The criteria also constituted an important linchpin in research looking at the genetics of JHS.

How reliable were the Brighton Criteria?

The Brighton criteria were validated in adults but not in children below the age of 16 years. The process of validation required 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 be 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).

What did the Brighton Criteria consist of?

The criteria have been reproduced (as published) below.

Revised diagnostic criteria for the Joint Hypermobility Syndrome (JHS)

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, 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.

JHS was diagnosed in the presence two major criteria, or one major and two minor criteria, or four minor criteria. Two minor criteria sufficed where there was an unequivocally affected first-degree relative.

JHS was excluded by presence of Marfan or Ehlers-Danlos syndromes. Criteria Major 1 and Minor 1 are mutually exclusive as are Major 2 and Minor 2.

 

Dr Alan J Hakim MA FRCP
Consultant Physician and Rheumatologist, & Chief Medical Advisor and Trustee, HMSA

Version 2 Updated March 2017

Planned review date March 2020

Academic Reference

Grahame R, Bird HA, Child A et al. The revised (Brighton 1998) criteria for the diagnosis of benign joint hypermobility syndrome (BJHS). J Rheumatol 2000 Jul;27(7):1777-9

Tinkle BT, Bird HA, Grahame R, Lavallee M, Levy HP, Sillence D. The lack of clinical distinction between hypermobility type of Ehlers-Danlos syndrome and the joint hypermobility syndrome. Am J Med Denet A. 2009; 149A(11):2368-70

Castori M, Tinkle B, Levy H, Grahame R, Malfait F, Hakim A. A framework for the classification of joint hypermobility and related conditions. Am J Med Genet C Semin Med Genet. 2017 Feb 1. doi: 10.1002/ajmg.c.31539. [Epub ahead of print] PubMed PMID: 28145606.

Tinkle B, Castori M, Berglund B, Cohen H, Grahame R, Kazkaz H, Levy H. 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 C Semin Med Genet. 2017 Feb 1. doi:10.1002/ajmg.c.31538.[Epub ahead of print] PubMed PMID: 28145611.


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