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Clinic Case. Alev Wilk, MD Primary Care Conference 4/28/04. No conflict of interest. Cases. 49 y.o. AA woman seen with a h/o multiple musculoskeletal symptoms Pain in lower back & neck, bilateral elbows & shoulders for months to years

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clinic case

Clinic Case

Alev Wilk, MD

Primary Care Conference


  • 49 y.o. AA woman seen with a h/o multiple musculoskeletal symptoms
    • Pain in lower back & neck, bilateral elbows & shoulders for months to years
    • PMH: Mild scoliosis, OA, lateral epicondylitis, TMJ dysfunction, left shoulder tendonitis, fibromyalgia
    • SH: Postal worker 5 yrs.; military service 9 yrs.
    • FH: possible Ehler-Danlos syndrome
    • PE: Musculoskeletal findings: hyperextension of MCP’s and thumbs; diffuse tenderness
    • Radiologic: osteoarthritic changes of the spine
  • 39 y.o. woman seen for intermittent low back pain:
    • 10-20 year h/o low back pain worse with prolonged sitting & standing
    • PMH: de Quervain’s tenosynovitis, olecranon bursitis, fibromyalgia, umbilical hernia
    • SH: cashier; gymnast as a teenager
    • FH: noncontributory
    • PE: musculoskeletal findings: marked elbow, knee, back extension, thumb apposition
    • Radiologic: retrolisthesis at L3-4, mild DJD
  • 27 y.o. woman seen for recurrent subluxation of her left shoulder
    • Several year h/o of left shoulder pain with recurrent subluxations
    • PMH: right wrist and shoulder tendonitis
    • SH: school teacher; rock climbing
    • FH: sister with repeated shoulder subluxations
    • PE: musculoskeletal: hyperextension at elbows and knees. Tenderness over supraspinatus tendon. Skin: striae, hyperextensibility
case summary
Case summary
  • Joint Hypermobility
  • Soft tissue injuries
  • Chronic pain
  • Osteoarthritis
  • Presentation, diagnosis, prevalence of joint hypermobility syndrome
  • Relationship to associated conditions (soft tissue injury, chronic pain, osteoarthritis)
  • Treatment of associated conditions when present
  • Musculoskeletal symptoms in presence of joint laxity
  • Heritable disorder of connective tissue
  • Identical to the hypermobility type of Ehler-Danlos syndrome (EDS type III)
  • Mechanism of injury: Increased ligamentous laxity; decreased joint proprioception
  • Nine-Point Beighton hypermobility score*
    • Passive dorsiflexion of the metacarpophalangeal joint to 90 degrees
    • Apposition of the thumb to the flexor aspect of the forearm
    • Hyperextension of the elbow to > 10 degrees
    • Hyperextension of the knee to > 10 degrees
    • Forward trunk flexion with hands flat on the floor and with knees extended

*Ann Rheum Dis 1973;32:413-7

revised diagnostic criteria
Revised diagnostic criteria
  • Major criteria
    • Beighton score of 4/9 or greater
    • Arthralgia for longer than 3 months in 4 or more joints
  • Minor criteria
    • Beighton score of 1,2 or 3/9
    • Arthralgia (>3 mo) in 1-3 joints, or back pain, spondylosis, spondylolysis/listhesis
    • Dislocation/subluxation in more than one joint, or in one joint on more than one occasion
    • Soft tissue rheumatism > 3 lesions
revised diagnostic criteria1
Revised diagnostic criteria
  • Minor criteria continued
    • Maranoid habitus (tall, slim, span/height > 1.03, arachnodactyly)
    • Abnormal skin: striae, hyperextensibility, thin skin, papyraceous scarring
    • Eye signs: drooping eyelids or myopia or antimongoloid slant
    • Varicose veins or hernia or uterine/rectal prolapse

2 major criteria or one major and 2 minor criteria; 4 minor criteria or 2 minor and an affected first-degree relative

  • 5-13% in childhood
  • 4-5% in the general population
  • 13-15% in rheumatology clinics
  • Higher rates reported in women
  • Higher rates reported in West Africans & Middle-Eastern women
benign condition
Benign Condition?
  • Heritable Disorder of Connective Tissue
  • Hypermobility syndrome
  • Benign Joint Hypermobility Syndrome
  • Mechanisms
    • Increased ligamentous laxity may lead to soft tissue pain/ligamentous injury
    • Decreased proprioception of hypermobile joints
associated conditions
Associated conditions
  • Tendency towards dislocation
  • Traumatic synovitis
  • Chronic joint pain
  • Fibromyalgia and depression
  • Premature osteoarthritis
bridges aj et al joint hypermobility in adults referred to rheumatology clinics
Bridges AJ et al. Joint hypermobility in adults referred to rheumatology clinics.
  • 130 adult patients referred to a rheumatology clinic with musculoskeletal problems
  • 97 women, 33 men
  • Beigton score > 5 out of 9
  • Exclusion criteria: signs of Ehler-Danlos syndrome (EDS)

Annals of the Rheumatic Diseases 1992;51:793-796

bridges aj et al results
Bridges AJ et al.Results
  • 15% had joint hypermobility
  • Average Beighton score of 8; average age of 50; hypermobile features since childhood
  • All cases of hypermobility were women
  • 65% had a FH of joint hypermobility in a first degree relative
  • Trunk hypermobility was noted in younger patients (average age of 37 y.o.)
bridges aj et al
Bridges AJ et al.
  • 50% had hypermobility of the fingers & ankles, 35% of the hips.
  • 85% flat feet
  • 35% scolioisis
  • 40% soft tissue injury
  • 60% OA
  • 30% fibromyalgia
hudson n et al diagnostic associations with hypermobility in rheumatology patients
Hudson N et al* Diagnostic associations with hypermobility in rheumatology patients.
  • 378 consecutive referrals to a rheumatology clinic
  • Beighton score of four or greater out of nine.
  • Assessed by physical exam and physical activity, leisure and work

Brit J of Rheumatology 1995;34:1157-61

hudson et al
Hudson et al.
  • 13.2% fulfilled criteria for hypermobility
  • 94% of the patients were women (73% in the control group); average age 45 y.o.
  • 26% had tendonitis, bursitis or fasciitis (17% in the control group)
  • 30% fit diagnositic criteria for fibromyalgia syndrome, the most frequent diagnosis (8% in the control group)
hudson et al1
Hudson et al.
  • 15% with axial or spinal complaints (15% in the control group)
  • 7% with OA (19% in the control group)
  • 4% with inflammatory arthropathy (32% in the control group)
hudson et al2
Hudson et al.
  • Pain breakdown:
    • Widespread in 44%
    • Multiple localized sites in 44%
    • Single site in 13%
    • Spinal-neck in 37%
    • Spinal-midthoracic & low back in 80%
    • Tendonitis (previous and present) in 63%
living with hypermobility
Living with Hypermobility*
  • Everyday activities carry the price of pain
    • ADL’s
    • Repetitive movement
  • Absenteeism from work
  • Adverse effects on family and relationships
  • Depression & isolation from pain and disability

*Rheumatology 2001;40:487-489

barriers to diagnosis and management
Barriers to diagnosis and management
  • Patients generally look well and present uniquely
  • Under-recognized and under-estimated
  • Lack of a clear cause-and-effect relationship
  • Non-specialized physical therapy can exacerbate symptoms
  • Specialized physiotherapy
    • Stabilizing lax joints with exercise to increase stability, reduce pain and diminish hypermobility
    • Improving proprioceptive acuity
  • Drug therapy
  • Cognitive-behavioral therapy
  • Patient support groups
  • Joint hypermobility appears to be associated with multiple soft tissue injuries, fibromyalgia and osteoarthritis
  • Joint hypermobility is relatively common in populations with & without musculoskeletal symptoms.
  • Joint hypermobility syndrome may be a true entity but like fibromyalgia is difficult to assess and treat.
  • Hudson N. Fitzcharles MA. Cohen M. Starr MR. Esdaile JM. The association of soft-tissue rheumatism and hypermobility. British Journal of Rheumatology. 37(4):382-6, 1998 Apr.
  • Acasuso-Diaz M. Collantes-Estevez E. Joint hypermobility in patients with fibromyalgia syndrome. Arthritis Care & Research. 11(1):39-42, 1998 Feb.
  • Hudson N, Starr MR, Esdaile JM, Fitzcharles MA. Diagnostic association hypermobility in Rheumatology Patients. British J of Rheumatology 1995;34:1157-1161.
  • Bridges AJ, Smith E, Reid J. Joint hypermobility in adults referred to rheumatology clinics. Annals of the Rheumatic Diseases 1992;51:793-796.
  • Gurley-Green S. Living with the hypermobility syndrome. Rheumatology 2001;40:487-489.
  • Grahame R, et al. The Revised (Brighton 1998) Criteria for the Diagnosis of Benign Joint Hypermobility Syndrome (BJHS). J of Rheumatology 2000;27:7.
  • Mishra MB, Ryan P, Atkinson P, et al. Extra-articular feature of benign joint hypermobility syndrome. Br J Rheumatol 1996;35:861-6.
  • Larsson LG, Mudholkar GS, Baum J, Srivastava DK. Benefits and liabilities of hypermobility in the back pain disorders of industrial workers. Journal of Internal Medicine 1995;238:461-467.
  • Larsson LG, Baum J, Mudholkar GS, Kollia GD. Benefits and disadvantages of joint hypermobility among musicians. N Engl J Med 1993;329:1079-81.
  • Fitzcharles MA. Is Hypermobility a Factor in Fibromyalgia. J of Rheumatology 2000;27:7.
  • Al-Rawi ZS, Al-Aszawi AJ, Al-Chalabi T. Joint mobility among university students in Iraq. Br J Rheumatol 1985;24:326-31.
  • Birrell FN, Adebajo AO et al. High prevalence of joint laxity in West Africans. Br J Rheumatol 1994;33:56-9.