Benign Joint Hypermobility Syndrome
Introduction[edit | edit source]
Joint Hypermobility Syndrome (JHS) was first described in 1967 by Kirk and colleagues as a condition where joint laxity is associated with various musculoskeletal complaints.[1] JHS encompasses various disorders such as Benign Joint Hypermobility Syndrome (BJHS), Ehler-Danlos Syndrome (EDS), Marfan Syndrome and Osteogenesis Imperfecta.[2] All of these disorders are classified as hereditary connective tissue disorders (HCTDs).[2]
Connective tissue is found throughout the body (ligaments, tendons, joints, the digestive tract, heart, eyes), so JHS does not just affect the joints. It can also affect skin extensibility, digestion and in more severe connective tissue disorders like Marfan Syndrome, it can affect heart function.[3]
Unlike other connective tissue disorders, BJHS is a condition which causes musculoskeletal symptoms in hypermobile patients, without other rheumatological features being present.[4] Its primary symptom is symptomatic hypermobility (ie excessive laxity) of multiple joints.[5][6] and it is associated with arthralgia, poor exercise tolerance and potentially recurrent subluxations.[7][5]
Epidemiology[edit | edit source]
Generalised joint laxity is often observed in people who do not have any other symptoms.[4] It has been found that hypermobility not associated with systemic disease occurs in 4% to 13% of the population.[4] In 1973, Beighton and colleagues demonstrated that the number of positive hypermobility tests was age and sex related - ie rates are higher in younger children and women have higher scores than age-matched men.[8] It also appears that rates vary across ethnicities,[8] with higher rates in Asian, African and Middle Eastern people.[4]
There have been a number of studies looking at prevalence rates of hypermobility in children. One study by Gedalia and colleagues found that generalised hypermobility occurs in 66% of school children with arthralgia of unknown cause.[4][9] However, while de Inocencio Arocena and colleagues found similar prevalence rates (hypermobility prevalence was 55%, and 71 % in children aged less than 8 years)[10] as Gedalia et al, they found that there was no association between hypermobility and developing arthralgia.[4][10] Thus, it appears that generalised hypermobility can exist without joint pain.[4]
BJHS has a strong genetic component and appears to be caused by an abnormality in collagen or the ratio of collagen subtypes.[4] Mutations in the fibrillin gene have been found in families with BJHS.[4]
- ↑ Kirk JA, Ansell BM, Bywaters EG. The hypermobility syndrome: musculoskeletal complaints look for more recent sources associated with generalized joint hypermobility. Ann Rheum Dis. 1967; 26(5): 419–25.
- ↑ 2.0 2.1 Neki NS, Chhabra A. Benign Joint Hypermobility Syndrome. Journal of Mahatma Gandhi Institute of Medical Sciences. 2016; 21(1): 12-18
- ↑ Prowse, T. Benign Joint Hypermobility Syndrome Course. Physioplus. 2020.
- ↑ 4.0 4.1 4.2 4.3 4.4 4.5 4.6 4.7 4.8 Simpson MMR. Benign Joint Hypermobility Syndrome: Evaluation, Diagnosis, and Management. J Am Osteopath Assoc. 2006;106(9): 531–536.
- ↑ 5.0 5.1 Ross J, Grahame R. Joint hypermobility syndrome. BMJ. 2011; 342: c7167.
- ↑ Russek LN. Hypermobility Syndrome, Physical Therapy. 1999; 79(6): 591-9.
- ↑ Magnusson SP, Julsgaard C, Aagaard P, Zacharie C, Ullman S, Kobayasi T et al.Viscoelastic properties and flexibility of the human muscle-tendon unit in benign joint hypermobility syndrome. The Journal of Rheumatology. 2001; 28(12): 2720-5.
- ↑ 8.0 8.1 Remvig L, Jensen DV, Ward RC. Epidemiology of general joint hypermobility and basis for the proposed criteria for benign joint hypermobility syndrome: review of the literature. The Journal of Rheumatology. 2007; 34(4): 804-9.
- ↑ Gedalia A, Brewer EJ Jr. Joint hypermobility in pediatric practice--a review. The Journal of Rheumatology.1993. 20(2): 371-374.
- ↑ 10.0 10.1 de Inocencio AJ, Ocaña CI, Benito OL. Laxitud articular: prevalencia y relación con dolor musculosquelético [Joint hypermobility: prevalence and relationship with musculoskeletal pain]. An Pediatr (Barc). 2004; 61(2):162-166.
