Benign Joint Hypermobility

Syndrome

Primary Care Conference

November 28, 2007
Rebecca L Byers
 Clinical Case
• Patient: 45 yo woman with 2 ½ month
history of right toe pain and one month of
bilateral sciatica, with R>L.
• Hit toe against furniture in late August.
Seen in UC and diagnosed with sprain.
Wore a padded boot for several weeks
with decrease in symptoms. Symptoms
returned with regular shoe use.
• Second visit – films of right toe showed
tiny fragment dorsal to first MTP joint. No
other abnormalities.
• Third visit – now has bilateral pain
radiating down both legs. L/S films show
mild DDD L4-L5, L5-S1 and 18 degrees of
convex-right scoliosis. Repeat toe films –
absence of prior fragment, dx of hallux
valgus.
• Exam: Easily places palms on the floor.
No vertebral tenderness, no SI joint
tenderness. Negative SLRs –
hyperflexible bilaterally (120 deg).
Tenderness over right first MTP, not red,
no swelling.
 Past Medical History
• Migraine headaches
• MVA 1997 with headache and neck pain;
resolved. Head CT negative. MRI neck
with small C6 bulging disk without nerve
root or spinal cord contact.
• Right breast cancer 2001, positive sentinel
node, 6/6 other nodes negative. Chemo x4
(adriamycin, cytoxan). Bilateral
mastectomy with reconstruction/implants.
 Joint Hypermobility
-ability to painlessly perform one of
the following maneuvers:

• 1. Extend the 5th metacarpophalangeal

joint more than 90 degrees, oppose the
thumb to the forearm (picture 1).
• 2. Extend the elbow more than 10
degrees beyond neutral (picture 2)
• 3. Extend the knee more than 10 degrees
beyond vertical (picture 3).
• 4. Place both palms on the floor without
bending the knees (picture 4).
 Beighton score
Specific joint laxity
Thumb to forearm, hyperextension of
fingers, hyperextension of elbow or knee,
palms to floor… Left, Right, Back

Nine anatomic sites. Calculation if one point

each with 9 being highest possible total.
 Hypermobility Joint Syndrome
• Disorder in which musculoskeletal pain
and generalized joint hypermobility occur
together.
• The term benign hypermobile joint
syndrome (BHJS) is used to distinguish
this non-life threatening disorder from
diseases such as the Marfan syndrome.
 Diagnostic criteria for BJHS
Major Criteria
• 1. Beighton score of 4/9 or greater
• 2. Arthralgia for more than 3 months in 4 or more joints.

Minor Criteria
• 1. A Beighton score of 1, 2 or 3/9 (0 to 3 if over age 50).
• 2. Arthralgia for 3 months or more in 1-3 joints, or back pain for 3
months or more, of spondylosis, spondylolysis, or spondylolisthesis.
• 3. Dislocation or subluxation in more than one joint, or in one joint
on more than one occasion.
• 4. Soft tissue rheumatism in 3 or more locations
(eg, epicondylitis, tenosynovitis, bursitis)
• 5. Marfanoid habitus.
• 6. Abnormal skin (eg, striae, hyperextensible,
thin or papyraceous scarring).
• 7. Eye abnormalities (eg, drooping eyelids,
myopia, anti mongoloid slant).
• 8. Varicose veins or hernia or uterine/rectal
prolapse.
 Pathogenesis
• Thought to be a disorder of collagen that
contributes to a loss of tensile strength,
with increased fragility of the joint capsule,
ligaments and tendons.
• High concordance in female monozygotic
twins (60%).
 Prevalence
• Generalized joint hypermobility – varies
from 10 to 30%. More common in the right
limb, females, blacks. Decreases with age.
• BHJS – prevalence is less certain. Most
studies done in Rheumatology clinics with
rates similar to Rheumatoid Arthritis (i.e.,
about 1% of the population).
 Clinical Manifestations
• Wide range of musculoskeletal complaints
-- brief joint “swelling”, symmetrical joint
pain during use and relieved with rest, and
arthralgias and myalgias without any
apparent abnormality.
-- back pain frequent; more common in
women: one study showed that 17 of 20
women without another cause of back pain
had joint laxity.
 Other Possible Associated
Symptoms
• Anxiety/Panic Attacks
• Cognitive Disorders
• Palpitations
• Shortness of breath
• Fatigue
• Orthostatic symptoms
Postulation of disordered autonomic nervous
system function due to chronic pain.
 References
• Beighton, PB, Grahame, R, Bird, HA.
Hypermobility of joints, 2nd ed, Springer, New
York 1989.
• Bridges, AJ, Smith, E, Reid, J. Joint
hypermobility in adults referred to rheumatology
clinics. Ann Rheum Dis 1992; 51:793.
• Gazit, Y, Nahir, AM, Grahame, R, Jacob, G.
Dysautonomia in the joint hypermobility
syndrome. Am J Med 2003; 115:33.
• Graham, R, Bird, HA, Child, A, et al. J
Rheumatol 2000; 27:1777.
• Hakim, AJ, Cherkas, LF, Grahame, R, et al. The
genetic epidemiology of joint hypermobility: a
population study of female twins. Arthritis
Rheum 2004; 50:2640.
• Sheon, RP, Moskowitz, RW, Goldberg, VM. Soft
Tissue Rheumatic Pain: Recognition,
Management, Prevention, 3rd ed, Williams &
Wilkins, Baltimore 1996.