Yackov Berkun, Hadassah Mnt Scopus

1. Rheumatic diseases in children Yackov Berkun, Hadassah Mnt Scopus

2. Juvenile rheumatic diseases • A wide range of conditions • Inflammatory, mechanical, behavioral or psychological • May present with symptoms localized to the musculoskeletal system • MS symptoms common in childhood • 15% occasional limb pains • 5% chronic MS pain, daily activities • Lasting for 3 months or more Berkun, JIA

3. Juvenile rheumatic diseases • Juvenile idiopathic Arthritis • Vasculitis • Kawasaki disease • Henoch-Schonlein purpura • Autoinflammatory • FMF • Panniculitis • CRMO/SAPHO syndromes • PFAPA • Connective tissue • SLE • Juvenile dermatomyositis • Inflammatory bowel disease • Mixed connective tissue disease • Linear scleroderma • Progressive systemic sclerosis

4. Mechanical/orthopedic • Infection/post-infective • Viral rubella, measles, mumps, parvo, hepatitis • Reactive arthritis • Rheumatic fever • Septic arthritis • Transient hip synovitis • Hematological • Idiopathic pain syndromes • Other Berkun, JIA

5. Introduction, JIA • Most frequent chronic inflammatory disease of childhood • One of the more common chronic illnesses of childhood and an important cause of disability • First series 1890, George Stil 1897 • Group of disorders defined by ILAR criteria 1997 Berkun, JIA

6. Epidemiology • All races and geographic areas • Prevalence: 0.1-4/1000 • Chronic disease /1000 • Epilepsy 3 • DM 1 • CP 1.3 • JIA 2 Berkun, JIA

7. Objectives • To recognize symptoms and signs of clinical subgroups of JIA • To understand laboratory and radiology studies helpful when considering the diagnosis • To be familiar with the differential diagnosis • To understand the general approaches to treatment Berkun, JIA

8. JRA American College of Rheumatology Revised Criteria • Age of onset < 16 years • Arthritis of one or more joints • Duration of disease > 6 weeks (EULAR 12) • Other conditions which present with arthritis in childhood must be excluded Berkun, JIA

9. ILAR classification Criteria, Durban 1997 • Oligoarthritis • persistent • extended • Systemic • Polyarticular RF+ • Polyarticular RF- • Psoriatic arthritis • Enthesitis-related arthritis (ERA) • Other Berkun, JIA

10. Oligoarthritis • Most common 40-60% of JIA • 4 or fewer joints in the first 6 months • Insidious onset • Age 1-3 years • Monoarthritis 50% • Large joints • Knee50%, ankle, elbow • Asymmetric • Systemic symptoms absent • Uveitis 30% Berkun, JIA

11. מפרק • נפיחות נוזל, היפרטרופיה, בצקת • ללא אודם • הגבלה בתנועה • כאב לא חד • ביטוים בתינוק Berkun, JIA

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16. Oligoarthritis, Laboratory • CBC, ESR normal • ANA 60-80% Berkun, JIA

17. 30% ANA Asymptomatic 50% at diagnosis Within 5 years Bilateral in 2/3 Loss of vision 10% Uveitis Berkun, JIA

18. Uveitis- hypopion, synechiae Berkun, JIA

19. Uveitis, Keratic precipitates Berkun, JIA

20. Uveitis, compl. Band kerathopathy Berkun, JIA

21. Uveitis, compl. Synechae Berkun, JIA

22. Slit lamp exam

23. Oligoarthritis, outcome Berkun, JIA

24. Oligoarthritis, outcome Berkun, JIA

25. ILAR classification Criteria, Durban 1997 • Oligoarthritis • persistent • extended • Systemic onset • Polyarticular RF+ • Polyarticular RF- • Psoriatic arthritis • Enthesitis-related arthritis (ERA) • Other Berkun, JIA

26. Systemic onset JIA • 10% of JIA • Female 40-50% • Prominent systemic symptoms • fever, rash, lymphadenopathy, hepatosplenomegaly, pericarditis, pleuritis • Arthritis may be absent for months to years • Uveitis uncommon Berkun, JIA

27. SoJIA, clinical features, fever Berkun, JIA Fever 2 weeks 390 Quotidian Spikes 1-2/d

28. Arthritis polyarticular • May be absent in 20% Berkun, JIA

29. Rash Berkun, JIA

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31. Rash Berkun, JIA

32. Koebner Berkun, JIA

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34. SoJIA, clinical features, rash • Evanescent nonfixed erythematous • Trunk • חיוורון היקפי • מופיעה עם חום • Koebner phenomena Berkun, JIA

35. SoJIA, clinical features, general • Serositis • Pericarditis Berkun, JIA

36. SoJIA, clinical features, general • Lymphadenopathy • Hepatosplenomegaly Berkun, JIA

37. Systemic JIA, criteria • Arthritis + fever > 2 wks • Quotidian > 3 days +1 of • evanescent nonfixed erythematous • generalized lymphadenopathy • hepato/ splenomegaly • serositis Berkun, JIA

38. SoJIA, laboratory • WBC   , Hgb, platelets  to   • ESR    • Ferritin    • ANA and RF negative Berkun, JIA

39. Complications • Heart tamponade • Malnutrition • Osteoporosis • Joint deformity • Amyloidosis • Growth retardation Berkun, JIA

40. Macrophage activation syndrome • Hemophagocyticlymphohistiocytosis • Life-threatening complication, cytokine storm • NK cells dysfunction • Sustained fever, hepatosplenomegaly, anemia, liver dysfunction, coagulopathy, CNS • Early diagnosis • Aggressive treatment Berkun, JIA

41. Disease courses • Persistent polyarthritis (50%) • Monocyclic, remission within 2–4 years (40%) • Relapsing polycyclic • Flares systemic + mild arthritis (10%) • Poor outcome • Systemic features> 0.5 years, thrombocytosis, polyarthritis incl. hip involvement • Mortality (2.8% to 14%) Berkun, JIA

42. ILAR classification Criteria, Durban 1997 • Oligoarthritis • persistent • extended • Systemic • Polyarticular RF- • Polyarticular RF+ • Psoriatic arthritis • Enthesitis-related arthritis (ERA) • Other Berkun, JIA

43. Polyarticular, fingers Berkun, JIA

44. Fingertenosynovitis Berkun, JIA

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46. Polyarticular disease, laboratory • WBC , Hgb, platelets WNL to  • ESR  to   • ANA 25% Berkun, JIA

47. Polyarthritis RF+ • 5-10% • Age >10 years • Symmetric • Rheumatoid nodules • Vasculitis • Lung Berkun, JIA

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