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BOARD REVIEW

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

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  1. BOARD REVIEW RHEUMATOLOGY Dennis A. Peacock April 9, 2008

  2. 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

  3. 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

  4. 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

  5. Labs cont. • ANA – 1:640 (diffuse pattern) • Left Foot X-ray – widening of the tibiofibular with soft-tissue swelling • Bone Scan – Left ankle inflammation

  6. 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

  7. 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

  8. 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

  9. 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)

  10. PICTURES

  11. 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

  12. 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)

  13. 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)

  14. Uveitis Pictures

  15. 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

  16. 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

  17. 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)

  18. Systemic JRA

  19. Systemic JRA • Associated with Serositis, Pleuritis, Pericarditis, Hyperbilirubinemia, elevated transaminases, anemia, leukocytosis, hepatosplenomegaly • 25% progress to chronic inflammatory arthritis • Usually ANA and RF negative

  20. 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)

  21. Differential Diagnosis • SLE • LUPUS • Rheumatic Fever • HSP • PAN • IBD • Viruses Joint hypermobility Reiter syndrome Reactive arthritis Psoriatic arthritis Enthesitis syndrome

  22. 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

  23. 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

  24. AnkylosingSpondylitis

  25. 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

  26. 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

  27. 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

  28. 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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