Instructions for applying Criteria

The BJHS is diagnosed in the presence of 2 major criteria, or one major and 2 minor criteria, or 4 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 syndrome [other than the EDS Hypermobility type (formerly EDS III) as defined by the Ghent 1996 and Villefranche 1998 Criteria, respectively]. Criteria Major 1 and Minor 1 are mutually exclusive, as are Major 2 and Minor 2.

Major Criteria

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

2) Arthralgia for longer than 3 months in 4 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 (≥ 3 months) in 1-3 joints or back pain (≥ 3 months), spondylosis, spondylolysis/spondylolisthesis

3) Dislocation/subluxation in more than 1 joint, or in one joint on more than one occasion.

4) Soft tissue rheumatism ≥ 3 lesions (e.g. Epicondylitis, tenosynovitis, bursitis).

5) Marfanoid habitus (tall, slim, span/height ratio > 1.03, upper: lower segment ratio < 0.89, arachnodactyly [+ Steinberg/wrist signs].

6) Abnormal skin: striae, hyper-extensibility, thin skin, papyraceous scarring

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

8) Varicose veins or hernia or uterine/rectal prolapse