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 |