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.

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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

benign hypermobility.10 However, defects in Tenascin X have

not been demonstrated in the vast majority of patients with
benign joint hypermobility. Prolidase is an enzyme involved in
collagen synthesis and catabolism of collagen and is primarily
involved in Proline recycling.11 Although patients with benign
hypermobility were demonstrated to have lower serum prolidase activity,11 it is not clear whether the relative deficiency
is the cause or a result of decreased collagen and connective
tissues. Unlike patients with heritable disorders of connective
tissue, subjects with benign hypermobility lack demonstrable
structural abnormalities in tendons and ligaments.12 Most
patients with symptomatic joint hypermobility have generalized or localized decrease in muscle bulk. Many patients
recall onset of symptoms after a systemic illness, suggesting
that loss of muscle mass and deconditioning might be
contributing to the clinical syndrome. Abnormalities of joint
proprioception are found in patients with symptomatic 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.

2.

Clinical manifestations

Patients with BJHS present with chronic or recurrent pain in

one more joints. In community studies, subjects with joint
hypermobility are more likely to have joint pains.5 Typically
patients with joint hypermobility have recurrent episodes of
joint pains or have joints pains precipitated by physical activity.13 The joint pains are a result of unrecognized microtrauma. Besides joint pains, patients present with enthesitis,
bursitis, tenosynovitis, chondromalacia patellae, rotator cuff
problems and mechanical back pain. Among patients with
soft tissue rheumatism complaints, presence of joint hypermobility has been linked to younger age at presentation and
recurrent or multiple lesions.14 Patients are at increased risk
of recurrent joint dislocations, subluxations and sprains as a
result of joint instability. 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. 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.15 Some patients may develop chronic low grade
synovitis as a consequence of recurrent low grade trauma
which may be misinterpreted as inflammatory arthritis.16
Several studies have noted the association of joint hypermobility with primary fibromyalgia. Patients with fibromyalgia have higher prevalence of hypermobility in comparison to
controls besides having higher mean Beighton scores.17,18
Interestingly the prevalence of hypermobility was higher
among patients who were diagnosed as fibromyalgia19 but did
not fulfil ACR 1990 criteria,20 suggesting that there might be
considerable overlap between the two conditions. Patient with
fibromyalgia may also be at increased risk of overuse syndromes. Among professional dancers, musculoskeletal complaints were more among those with hypermobility than
others despite equivalent training.21 Hypermobile subjects

have lower bone mineral density compared to controls,22

which predispose them to fractures. Besides bones and
joints, these patients may have other extra articular manifestations such as mitral valve prolapse,23 hernias24 and prolapse of rectum25 and uterus. Diminished stiffness of
vasculature predisposes to risk of varicose veins26 and eye
involvement may lead to high myopia.

3.

Diagnosis

Joint hypermobility is assessed at nine genetically determined

sites for the modified Beighton score (Table 1).1,27 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. A revised
(Brighton 1998) criteria1,28 has been proposed for the classification of BJHS (Table 2). Evaluation should be directed at
exclusion of other heritable disorders of connective tissues
that are associated with hypermobility including Ehlers Danlos syndrome, Osteogenesis imperfecta and Marfan's syndrome2 besides other systemic diseases associated with joint
pains including malignancies or rheumatological diseases like
fibromyalgia.13 Although a score of 4/9 or more is considered
significant, some patients may have symptoms with fewer
joints involved. However since hypermobility trait is very
common in several populations including India, other causes
should be actively sought before attributing the symptoms to
joint hypermobility.

4.

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. Sympathetic counselling and reassurance
goes a long way towards relieving anxiety and reducing maladaptive behaviour. Avoidance of physical activity and exercise leads to further decreased muscle mass and
deconditioning. Targeted exercise therapy is therefore the
mainstay of management of BJHS. Patients with hypermobility syndrome have decreased joint proprioception
compared to normal population.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.
Weakness of stabilizer muscles is a common finding among

Table 1 e Modified Beighton score. Hypermobility present

if total score >3.
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

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.
Major criteria
Beighton score more than or equal to 4
Arthralgia in >3 months in >4 joints.
Minor criteria
Beighton score of 1e3
Arthralgia >3 months in 1e3 joints or back pain >3 months
Spondylosis, spondylolysis, or spondylolisthesis
Soft tissue rheumatism lesions >3 (epicondylitis, tenosynovitis,
bursitis)
Marfanoid habitus ( armspan to height ratio >1.03 or upper
segment: lower segment ration <0.89
Abnormal skin striae
Eye signs
Varicose veins
BJHS 2 major or one major plus 2 minor or 4 minor
Beighton score and arthralgia to be counted only once
2 minor sufficient if first degree relative has hypermobility
syndrome
Exclusions: Ehlers Danlos other than type III (hypermobility type)
and Marfans syndrome

patients with joint hypermobility and pains. Vastus medialis

oblique and multifidus are examples of stabilizer muscles of
knee and spine respectively and their weakness is implicated
in anterior knee pain and mechanical low back ache.
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.
The objective of the exercise therapy is to strengthen and
stabilize the hypermobile joints and thereby protect the joints
from micro trauma.31 Exercises designed to improve proprioception decreased knee pain in cohort studies as well as in a
randomized controlled study.29,31,32 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, functional status and parents
global assessment, but targeted exercise was superior to
generalized exercise for improving parents global assessment
of improvement.33 Among exercises, closed kinetic chain exercises are most useful in improving joint stability and proprioception.1,32 The exercising limb is visualized as a chain of
jointed segments, the kinetic chain. In closed kinetic chain,
the ends of the chain are fixed (closed) while intervening
segments make small controlled movements.1 Back pain
benefits from regular walks and improved posture. Exercise
therapy should be under the supervision of experienced
physiotherapists familiar with the condition as inappropriate
exercises may increase joint instability and worsen symptoms. Despite the evidence of increased musculoskeletal
symptoms among subjects with joint hypermobility, the vast
majority remain asymptomatic and might indeed become an
asset in certain professions like music,34 ballet and dancing.35

Conflicts of interest
The author has none to declare.

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