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i n d i a n j o u r n a l o f r h e u m a t o l o g y 9 ( 2 0 1 4 ) S 3 3 eS 3 6

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Review Article

Benign joint hypermobility syndrome
Able Lawrence
Department of Clinical Immunology, SGPGIMS, Lucknow, India

abstract
Keywords:

Benign joint hypermobility syndrome is the presence of musculoskeletal symptoms in

Benign joint hypermobility syn-

subjects with joint hypermobility in the absence of demonstrable systemic rheumatic

drome

disease. Unlike the heritable disorders of connective tissue with which it shares consid-

Therapeutic exercise

erable overlap in manifestations, most subjects with hypermobility remain asymptomatic.
Prevalence of hypermobility varies considerably with age, gender and ethnicity. Muscle
weakness and decreased proprioception contribute to recurrent micro trauma and joint
pains in this otherwise benign condition. Supervised exercises designed to improve joint
stability and proprioception remain the mainstay of treatment.
Copyright © 2014, Indian Rheumatology Association. All rights reserved.

1.

Introduction

Exaggerated flexibility of joints beyond the normal range occurs in certain individuals and had been recognised from
ancient times.1 While joint hypermobility is a feature of
several inherited diseases of connective tissue such as
Osteogenesis imperfecta, Marfan's syndrome and Ehlers
Danlos syndrome,2 it is also present in a subset of otherwise
normal individuals. Joint hypermobility in such normal subjects is termed benign to distinguish it from the more serious
heritable disorders of connective tissue.3 Presence of musculoskeletal symptoms in subjects with hypermobility in the
absence of demonstrable systemic disease is termed benign
joint hypermobility syndrome. However, hypermobile type
Ehlers Danlos syndrome can overlap and may be indistinguishable from benign hypermobility syndrome.3
Prevalence of joint hypermobility varies based on age, sex
and ethnicity. It is common in children and decreases with
age.4,5 Men have less hypermobility than women.6 There are
wide variations between different ethnic groups across and

within regions. While Caucasian boys and girls have 12.9%
and 40.5% prevalence of hypermobility, among adults it is only
10%. While Nigerian undergraduate students had hypermobility of 12.9% (8% in males and 17% in females),7 the prevalence was higher among the Yoruba population in south
western Nigeria at 43% (35% in males and 57% in females).8 In
a study among children aged 3 to 19 from Mumbai, 58% had
hypermobility5 while 91% medical students from Kerala had
Beighton score of 4/9 or more1 underscoring the wide variation
in the prevalence of hypermobility between different communities. Despite the widely varying prevalence of hypermobility as a trait in different communities, and the higher
rates of musculoskeletal pains, the vast majority are asymptomatic5 highlighting the benign nature of the condition.
Unlike heritable disorders of connective tissue (HDCT), the
biochemical aetiology of benign joint hypermobility syndrome
(BJHS) is unknown. Despite its benign nature in comparison
with the HDCT, there is considerable clinical overlap.9 While
the vascular type Ehlers Danlos syndrome due to Tenascin X
deficiency is autosomal recessive, heterozygous carriers have
variable amount of joint hypermobility indistinguishable from

E-mail address: abledoc@gmail.com.
http://dx.doi.org/10.1016/j.injr.2014.09.009
0973-3698/Copyright © 2014, Indian Rheumatology Association. All rights reserved.

S34 i n d i a n j o u r n a l o f r h e u m a t o l o g y 9 ( 2 0 1 4 ) S 3 3 eS 3 6 benign hypermobility. 2. Patients with hypermobility syndrome have decreased joint proprioception compared to normal population. Assessment site Hyperextension of elbow >10 Thumb touching the forearm Hyperextension of 5th MCP joint >90 Hyperextension of knee joint >10 Palm of hands touching flat on the ground with knees extended Right Left 1 1 1 1 1 1 1 1 1 . suggesting that loss of muscle mass and deconditioning might be contributing to the clinical syndrome. 4. In community studies. Prolidase is an enzyme involved in collagen synthesis and catabolism of collagen and is primarily involved in Proline recycling. Abnormalities of joint proprioception are found in patients with symptomatic joint hypermobility. Among professional dancers. patients present with enthesitis. Management of BJHS Most patients with benign joint hypermobility are concerned and apprehensive about their illness and would be relieved to know that they do not suffer from any systemic illness requiring therapy. defects in Tenascin X have not been demonstrated in the vast majority of patients with benign joint hypermobility. Joint hyperlaxity and diminished joint proprioception coupled with poor muscle bulk and tone may contribute to the risk of recurrent microtrauma and joint pains.10 However.22 which predispose them to fractures. Patients with fibromyalgia have higher prevalence of hypermobility in comparison to controls besides having higher mean Beighton scores. Clinical manifestations Patients with BJHS present with chronic or recurrent pain in one more joints. other causes should be actively sought before attributing the symptoms to joint hypermobility.23 hernias24 and prolapse of rectum25 and uterus. Patient with fibromyalgia may also be at increased risk of overuse syndromes.21 Hypermobile subjects have lower bone mineral density compared to controls. Unlike patients with heritable disorders of connective tissue. subluxations and sprains as a result of joint instability.16 Several studies have noted the association of joint hypermobility with primary fibromyalgia.5 Typically patients with joint hypermobility have recurrent episodes of joint pains or have joints pains precipitated by physical activity. subjects with benign hypermobility lack demonstrable structural abnormalities in tendons and ligaments. rotator cuff problems and mechanical back pain. 3. Osteogenesis imperfecta and Marfan's syndrome2 besides other systemic diseases associated with joint pains including malignancies or rheumatological diseases like fibromyalgia. presence of joint hypermobility has been linked to younger age at presentation and recurrent or multiple lesions. Avoidance of physical activity and exercise leads to further decreased muscle mass and deconditioning. However since hypermobility trait is very common in several populations including India. Diminished stiffness of vasculature predisposes to risk of varicose veins26 and eye involvement may lead to high myopia. Hypermobility is associated with higher risk of postural or mechanical back pain in professions that require prolonged sitting or standing while it is protective for those who have to frequently change positions. Besides bones and joints. chondromalacia patellae. Targeted exercise therapy is therefore the mainstay of management of BJHS. these patients may have other extra articular manifestations such as mitral valve prolapse.28 has been proposed for the classification of BJHS (Table 2).13 Although a score of 4/9 or more is considered significant.11 Although patients with benign hypermobility were demonstrated to have lower serum prolidase activity.27 A diagnosis of BJHS is made when the patient has pain in multiple joints along with generalized hypermobility (Beighton > 3) and other secondary causes of joint pain are excluded. some patients may have symptoms with fewer joints involved. Hypermobility present if total score >3. musculoskeletal complaints were more among those with hypermobility than others despite equivalent training.12 Most patients with symptomatic joint hypermobility have generalized or localized decrease in muscle bulk.13 The joint pains are a result of unrecognized microtrauma. A revised (Brighton 1998) criteria1. Among patients with soft tissue rheumatism complaints. Evaluation should be directed at exclusion of other heritable disorders of connective tissues that are associated with hypermobility including Ehlers Danlos syndrome.18 Interestingly the prevalence of hypermobility was higher among patients who were diagnosed as fibromyalgia19 but did not fulfil ACR 1990 criteria. Many patients recall onset of symptoms after a systemic illness.29 The muscles around joints can be classified into stabilizers close to the inside responsible for proper alignment and stability and prime movers providing the strength for different active movements. tenosynovitis. Diagnosis Joint hypermobility is assessed at nine genetically determined sites for the modified Beighton score (Table 1). Besides joint pains.15 Some patients may develop chronic low grade synovitis as a consequence of recurrent low grade trauma which may be misinterpreted as inflammatory arthritis. Weakness of stabilizer muscles is a common finding among Table 1 e Modified Beighton score. Some patients may develop correctible deformities of joints without ever suffering from arthritis1 and sometimes correctible deformities can result from transient and self-limited arthritis.1.11 it is not clear whether the relative deficiency is the cause or a result of decreased collagen and connective tissues.17. Sympathetic counselling and reassurance goes a long way towards relieving anxiety and reducing maladaptive behaviour. subjects with joint hypermobility are more likely to have joint pains.14 Patients are at increased risk of recurrent joint dislocations. bursitis.20 suggesting that there might be considerable overlap between the two conditions.

37:382e386. Minor criteria Beighton score of 1e3 Arthralgia >3 months in 1e3 joints or back pain >3 months Spondylosis. Mudholkar GS. 2012. 2005. when and how does joint hypermobility lead to osteoarthritis? Br J Rheumatol. J Intern Med.15:75.33:160e172. Hypermobility: features and differential incidence between the sexes. Benign hypermobiligy syndrome. 17. Grahame R. Karaaslan Y. Starr MR. Engelbert RH. 2014. 5. Clin Rheumatol.31. Kul-Panza E. Guven Z. Srivastava DK.29.31 Exercises designed to improve proprioception decreased knee pain in cohort studies as well as in a randomized controlled study.33:56e59.50:2742e2749. 19. closed kinetic chain exercises are most useful in improving joint stability and proprioception. Larsson LG. the ends of the chain are fixed (closed) while intervening segments make small controlled movements. de Vos R.25:291e293. 2.35 Conflicts of interest The author has none to declare. 2006. De Paepe A. Approach to the patient with noninflammatory musculoskeletal pain. Mudholkar GS. BMC Musculoskelet Disord. J Indian Rheumatol Assoc. In closed kinetic chain. Scheper MC. Shenoi S. de Vries JE. Joint hypermobility and primary fibromyalgia: a clinical enigma. Munns C. Abnormal joint posture has been found in patients with joint hypermobility and pain30 and the excess mobility coupled with decreased proprioception contributes to micro trauma during physical activity.1. Dapper DV. FASEB J. The genetic basis of the joint hypermobility syndromes. Grahame R.26:146e150. Hakim AJ. Larsson LG. Silman AJ. Harvey W.32 The exercising limb is visualized as a chain of jointed segments. 15. 1989. Yunus MB. Em S. Pediatr Clin North Am. Couppe C. Hazleman BL.34 ballet and dancing. Fitzcharles MA. et al. Ozturk M. 18. the vast majority remain asymptomatic and might indeed become an asset in certain professions like music. Grahame R. Ann Rheum Dis. Elliott EJ. Nollet F. Gurer G. Gunduz OH. Jensen JK. Ucar D. 21. How often. 14. Rheumatology (Oxford).32 Kemp et al compared generalized exercise with joint targeted exercise among children with joint hypermobility and pain and found both equally useful in reducing pain. 9. Constine L. 6. Sendur OF. Silverman S. Hypermobility in women with fibromyalgia syndrome. Nielsen RH. The American College of Rheumatology 1990 criteria for the classification of fibromyalgia.45:502e507. Arthritis Rheum. East Afr Med J. Arthritis Rheum. Clin Exp Rheumatol. or spondylolisthesis Soft tissue rheumatism lesions >3 (epicondylitis. Pacey V. Clin Rheumatol. . 2008. spondylolysis. et al. Hakim AJ.34:177e180. Didia BC. but targeted exercise was superior to generalized exercise for improving parents global assessment of improvement. 1975. Pediatr Rheumatol Online J. Generalized joint hypermobility in professional dancers: a sign of talent or vulnerability? Rheumatology (Oxford).30:1426e1430. bursitis) Marfanoid habitus ( armspan to height ratio >1. Exercise therapy should be under the supervision of experienced physiotherapists familiar with the condition as inappropriate exercises may increase joint instability and worsen symptoms. Esdaile JM. Low tendon stiffness and abnormal ultrastructure distinguish classic EhlersDanlos syndrome from benign joint hypermobility syndrome in patients. Malfait F. 2014 Aug 13.i n d i a n j o u r n a l o f r h e u m a t o l o g y 9 ( 2 0 1 4 ) S 3 3 eS 3 6 Table 2 e Revised Brighton 1998 criteria for Benign joint hypermobility.26:485e487. Birrell FN. High prevalence of joint laxity in West Africans. The objective of the exercise therapy is to strengthen and stabilize the hypermobile joints and thereby protect the joints from micro trauma.7:1. the kinetic chain. tenosynovitis. Schalkwijk J. The association of soft-tissue rheumatism and hypermobility. Tofts LJ. pii: fj.59:471e492. S35 references 1. Baum J. Joint hypermobility in Indian children. 8. 1995. 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