1 / 28

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

paul-maldonado
Télécharger la présentation

BOARD REVIEW

An Image/Link below is provided (as is) to download presentation Download Policy: Content on the Website is provided to you AS IS for your information and personal use and may not be sold / licensed / shared on other websites without getting consent from its author. Content is provided to you AS IS for your information and personal use only. Download presentation by click this link. While downloading, if for some reason you are not able to download a presentation, the publisher may have deleted the file from their server. During download, if you can't get a presentation, the file might be deleted by the publisher.

E N D

Presentation Transcript

  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

More Related