Benign Joint Hypermobility Syndrome
Benign Joint Hypermobility Syndrome (BJHS) is a condition that comes from the Rheumatology literature.[1] It is thought by many to be the same condition as hypermobile Ehlers Danlos Syndrome, a condition that comes from the Genetics and Paediatrics literature.
Hypermobility vs. Joint Hypermobility Syndrome
Hypermobility is fairly common. This is especially so in younger people, women, and in those with Asian and African descent. Most hypermobile people are asymptomatic and don't have an underlying connective tissue disorder.
Joint hypermobility syndrome is a heritable connective tissue disorder that is characterised by chronic pain, musculoskeletal dysfunction such as dislocations, chronic fatigue, dysautonomia, amongst other problems.
Diagnosis
The diagnosis of Benign Joint Hypermobility Syndrome (BJHS) is made with the 2000 Brighton criteria (not to be confused with the Beighton score).[1]
- Major Criteria
- Beighton score of โฅ 4
- Arthalgia for longer than 3 months in 4 or more joints
- Minor Criteria
- Beighton score of 1, 2, or 3
- Arthalgia (> 3 month duration) in one to three joints or back pain (> 3 month duration) or spondylosis, spondylolysis/spondylolisthesis
- Dislocation or subluxation in more than one joint, or in one joint on more than one occasion
- Three or more soft tissue lesions (eg, epicondylitis, tenosynovitis, bursitis)
- Marfanoid habitus: tall, slim, arm span greater than height >1.03 ratio, upper segment less than lower segment <0.89 ratio, arachnodactyly
- Skin striae, hyperextensibility, thin skin, or abnormal scarring
- Ocular signs: drooping eyelids, myopia, antimongoloid slant
- Varicose veins, hernia, or uterine or rectal prolapse
- Mitral valve prolapse
- Requirements for diagnosis are any one of the following:
- Two major criteria
- One major plus two minor criteria
- Four minor criteria
- Two minor criteria and unequivocally affected first-degree relative in family history