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BOARD REVIEW. RHEUMATOLOGY Dennis A. Peacock April 9, 2008. CASE PRESENTATION. 8 month old male with swollen left ankle for several days No Fever and no signs of being ill Normal growth and development. Physical Exam. Alert, Playful, NAD

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Board review

BOARD REVIEW

RHEUMATOLOGY

Dennis A. Peacock

April 9, 2008


Case presentation
CASE PRESENTATION

  • 8 month old male with swollen left ankle for several days

  • No Fever and no signs of being ill

  • Normal growth and development


Physical exam
Physical Exam

  • Alert, Playful, NAD

  • Left ankle and dorsum of the foot is markedly swollen

  • Left ankle is erythematous & warm

  • Tender to palpation and movement

  • Limited Range of Motion

  • 2nd toe with edema and erythema but non-tender and full ROM


LABS

  • WBC – 9.1 52% segs, 40% lymphs, 7% monos, 1% eos

  • H/H – 12/36, Plts – 454

  • ESR – 69

  • U/A – normal

  • ASO – negative

  • ParvoB19 – negative

  • Lyme titers – negative


Labs cont
Labs cont.

  • ANA – 1:640 (diffuse pattern)

  • Left Foot X-ray – widening of the tibiofibular with soft-tissue swelling

  • Bone Scan – Left ankle inflammation


Case continued
Case Continued

  • Two weeks later signs of arthritis are seen in both ankles, both knees, and both wrists

  • WHAT IS THE DIAGNOSIS?

    • JUVENILE RHEUMATOID ARTHRITIS


JRA

  • Basics

    • Most common rheumatic dz in children

    • Incidence ~14 per 100,000

    • Prevalence ~115 per 100,000

    • First described by George Still in 1897


Diagnosis of jra
Diagnosis of JRA

  • Criteria

    • Age at onset <16

    • Arthritis

    • At least 6 weeks duration

    • Type defined by disease in 1st 6 months

      • Polyarthritis: 5 or more joints

      • Oligoarthritis: <5 inflammed joints

      • Systemic: characteristic fever

    • Exclusion of other types of arthritis


Major presentations of jra
Major Presentations of JRA

  • Joint effusions/swelling/pain

  • Loss of normal anatomic landmarks

  • Gradual onset of symptoms

  • Morning Stiffness

  • Gel phenomeon

    • Napping and Prolonged sitting after inactivity (Stiffness)



Oligoarthritis pauciarticular
Oligoarthritis (Pauciarticular)

  • Inflammation of 4 or fewer joints

  • ~60% of all cases of JRA

  • Two sub-classes

    • Early-onset Pauciarticular (EOPA)

      • Female to male predominance

      • Onset usually before 5 years of age

      • Usually ANA+ in young females

      • Rheumatoid Factor Negative

      • Strong association with Uveitis/Iridocyclitis


Uveitis
Uveitis

  • 50% with EOPA have chronic asymptomatic iridocyclitis

    • Inflammation of Iris and ciliary body

  • Untreated uveitis may develop synechiae (adhesions) between iris and lens

  • May progress to band keratopathy (calcium deposits in cornea)


Uveitis1
Uveitis

  • Must adhere to guidelines for exams

    • Oligo and Polyarticular disease  ANA+ and onset < 7y are at greatest risk

      • Must examine eyes every 3-4 months

    • Oligo and Polyarticular disease that are ANA- or onset > 7y are at medium Risk

      • Examine eyes every 6 months

    • Systemic are at least risk (q 12 months)



Oligoarthritis
Oligoarthritis

  • Late Onset Pauciarticular (LOPA)

    • Generally affects boys > 8 years old

    • Involves Hips/SI joints, Knees, Ankles, Feet (especially Achilles Tendinitis)

    • Associated with family history of spondyloarthropathies or psoriasis

    • May be associated with HLA-B27 +

    • May progress to be categorized as other spondyloarthropathies


Polyarthritis
Polyarthritis

  • 5 or more joints

  • 30% of all cases of JRA

  • Females > Males

  • Less associated with extraarticular involvement

  • Usually RF –

  • May be ANA+ (especially if RF+)  associated with worse disease and joint destruction


Systemic onset jra
Systemic Onset JRA

  • Still’s Disease (~15% cases JRA)

  • Males > Females (?)

  • Characterized by fever, rash, irritability, arthritis, and visceral involvement

  • Fever (>39) that occurs twice daily

  • Rash: 2-6mm, evanescent, salmon colored (trunk/proximal extremeties)



Systemic jra1
Systemic JRA

  • Associated with Serositis, Pleuritis, Pericarditis, Hyperbilirubinemia, elevated transaminases, anemia, leukocytosis, hepatosplenomegaly

  • 25% progress to chronic inflammatory arthritis

  • Usually ANA and RF negative


Differential diagnosis
Differential Diagnosis

  • Often Seronegative so often a disease of exclusion

  • Must differentiate between Septic arthritis

  • Must exclude lyme disease (may mimic oligoarthritis

  • Distinguish between Leukemia and JRA (bone pain v. joint pain)


Differential diagnosis1
Differential Diagnosis

  • SLE

  • LUPUS

  • Rheumatic Fever

  • HSP

  • PAN

  • IBD

  • Viruses

Joint hypermobility

Reiter syndrome

Reactive arthritis

Psoriatic arthritis

Enthesitis syndrome


Treatment of jra
Treatment of JRA

  • Anti-inflammatory

    • NSAIDS

      • Obviously risk of GI bleed, increased LFT’s, Reye-like syndrome or encephalopathy

    • Immunosuppressives (Steroids)

      • Used if severe disease

      • Cardiac Involvement

    • New immunomodulators

      • Enbrel


Spondyloarthropathies
Spondyloarthropathies

  • JuvenilleAnkylosingSpondylitis

    • Male:female ratio of 3:1

    • Affects Axial Joints (SI JOINT!!)

      • Ossification of anterior spinal ligament and fusion of the facets “bamboo spine”

      • Night pain/morning stiffness/pain with rest

    • ANA/RF negative

    • HLA-B27 + in 90%

    • Can be associated with uveitis/iritis


Ankylosing spondylitis
AnkylosingSpondylitis


Spondyloarthropathies1
Spondyloarthropathies

  • Reiter’s Syndrome

    • “Can’t see, can’t pee, can’t climb a tree”

    • Urethritis, Iritis, Arthritis

    • Post-infectious (1-3 weeks after)

      • Enteric pathogens (yersinia, shigella, salmonella)

      • Non-gonococcalurethritis (Chlamydia)

    • NSAIDS and antibiotics to treat underlying illness

    • May be associated with HLA-B27


Spondyloarthropathies2
Spondyloarthropathies

  • IBD

    • Chron’s and UC may be associated with arthritis

    • Tends to affect limb joints

    • May be associated with HLA-B27 and spine involvement

    • Affects about 1 in 5 with IBD

    • Treat underlying disease


Joint hypermobility
Joint Hypermobility

  • Increased mobility of joints

    • >10° hyperextension elbows/knees

    • Thumb to forearm… etc.

  • May be associated with Ehlers-danlos

  • Increased risk of dislocations and joint pains

  • Treat with NSAIDS

  • Reassure Parents


Functional joint complaints
Functional Joint Complaints

  • Growing Pains

    • Likely due to overuse of muscles/joints

    • Occur later in the day

    • Feels better with touch/massage

    • Girls more likely to complain of pains

    • Normal growth/development

    • Pain does not awaken from sleep

    • Treat with supportive care  massage, heating pad, stretching, NSAIDS


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