Diagnostic criteria for BJHS

Diagnostic criteria for benign joint hypermobility syndrome

• 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 one 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, hyperextensibility, thin skin, papyraceous scarring
  • 7) eye signs: drooping eyelids or myopia or antimongoloid slant
  • 8) varicose veins or hernia or uterine/rectal prolapse

Notes:

• benign joint hypermobility syndrome (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
• BJHS is excluded by the presence of Marfan's or Ehlers–Danlos syndrome (EDS) [other than the EDS Hypermobility type (formerly EDS III)]
• Criteria Major 1 and Minor 1 are mutually exclusive, as are Major 2 and Minor 2.