Table 2.

The Brighton criteria for the diagnosis of BJHS (adapted from Graham et al. [17]

Major criteria

1. A Beighton score of 4/9 or greater (either currently or historically)

2. Arthralgia for longer than 3 months in four or more joints.

Minor criteria.

1. A Beighton score of 1,2, or 3/9 (0,1,2,or 3 if aged 50+).

2. Arthralgia (for 3 months or longer) in one to 3 joints or back pain for (for 3 months or longer), spondylosis, spondylolysis/spondylolisthesis.

3. Dislocation/subluxation in more than one joint, or in one joint on more than one occasion.

4. Soft tissue rheumatism: three or more lesions (e.g., epicondylitis, tenosynovitis, bursitis).

5. Marfanoid habitus (tall, slim, span/height ration >1.03 upper: lower segment ration less than 0.89, arachnodactyly (positive steinberg/wrist signs).

6. Abnormal skin striae, hyperextensibility, thin skin, papyraceous scarring.

7. Eye signs: drooping eyelids or myopia or antimongoloid slant.

8. Varicose veins or hernia or uterine/rectal proplapse.

BJHS is diagnosed in the presence of 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.