The Diagnostic Criteria for Joint Hypermobility Syndrome (JHS)
Why is 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 use the criteria to confidently make the diagnosis of JHS. It is 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 constitute an important linchpin in research looking at the genetics of JHS.
How reliable are the Brighton Criteria?
The Brighton 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 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 are both very high (93% each).
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.
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. Criteria Major 1 and Minor 1 are mutually exclusive as are Major 2 and Minor 2.
Is there a difference between Joint Hypermobility Syndrome and Ehlers Danlos Syndrome?
The most important thing to appreciate is that the investigations, management, and advice are exactly the same for both JHS and EDS-HM.
Joint Hypermobility Syndrome (JHS) and Ehlers Danlos Syndrome – Hypermobility Type (EDS-HM) are the most common of the hypermobility conditions. The terminology can get a little confusing because some experts will use them inter-changeably. Also EDS-HM was formerly called EDS-Type III, but the numbering nomenclature for the various types of EDS has changed.
The Brighton criteria for diagnosing JHS arose from recognizing an association between hypermobility, injury to joints and soft tissues around joints, and pain. Initially it included things like mild variants of the skin changes described above, and the often-found body shape called the marfanoid habitus. It then developed further over the last decade as associations with, for example, bowel, autonomic cardiovascular, and pelvic floor problems became clear. These new findings, however, are not yet incorporated into the formal criteria for JHS.
The criteria for EDS-HM are based on observation that one can find minor clinical features of the more rare variants of EDS in some people and that they are also hypermobile and may experience pain. These might include clinical findings such as severe dislocations, more florid skin signs and in particularly its elasticity, a large curvature of the spine (a scoliosis), prolapse of the mitral valve of the heart, or association with dental or gum disease. However, these patients will also exhibit the same findings as those described for JHS.
Also, currently there is no genetic test that can identify either condition or separate them.
Therefore, some experts now consider JHS and EDS-HM to be the same thing albeit derived from different clinical perspectives. Whether EDS-HM or JHS is diagnosed is a clinical judgement. There is no absolute medical consensus on this matter. A doctor might, for example, use one term rather than the other dependent on the type and severity of issues present. EDS-HM might be the preferred name to give an individuals’ condition if there are findings such as those described in the main article in this section (severe dislocations, more florid skin signs, etc.). However, again only for example, the term JHS might be the preferred term if the principle issue is one of joint pain around hypermobile non-dislocating joints with few other signs.
Dr Alan J Hakim MA FRCP
Consultant Physician and Rheumatologist
Hon. Senior Lecturer
Barts Health NHS Trust and Queen Mary University London
Updated: June 2013. Planned date of review 2016
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