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Clinic Case

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

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  1. Clinic Case Alev Wilk, MD Primary Care Conference 4/28/04

  2. No conflict of interest

  3. 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 • 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

  4. Cases • 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

  5. Cases • 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

  6. Case summary • Joint Hypermobility • Soft tissue injuries • Chronic pain • Osteoarthritis

  7. Objectives • Presentation, diagnosis, prevalence of joint hypermobility syndrome • Relationship to associated conditions (soft tissue injury, chronic pain, osteoarthritis) • Treatment of associated conditions when present

  8. Presentation • 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

  9. Diagnosis • 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

  10. 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

  11. 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

  12. Prevalence • 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

  13. 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

  14. Associated conditions • Tendency towards dislocation • Traumatic synovitis • Chronic joint pain • Fibromyalgia and depression • Premature osteoarthritis

  15. 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

  16. 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.)

  17. 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

  18. 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

  19. 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)

  20. 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)

  21. 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%

  22. 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

  23. 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

  24. Treatment • 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

  25. Summary • 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.

  26. References • 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.

  27. References • 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.

  28. References • 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.

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