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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

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Clinic case

Clinic Case

Alev Wilk, MD

Primary Care Conference

4/28/04


Clinic case

No conflict of interest


Cases

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


Cases1

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


Cases2

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


Case summary

Case summary

  • Joint Hypermobility

  • Soft tissue injuries

  • Chronic pain

  • Osteoarthritis


Objectives

Objectives

  • Presentation, diagnosis, prevalence of joint hypermobility syndrome

  • Relationship to associated conditions (soft tissue injury, chronic pain, osteoarthritis)

  • Treatment of associated conditions when present


Presentation

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


Diagnosis

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


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


Prevalence

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


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


Treatment

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


Summary

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.


References

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.


References1

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.


References2

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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