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Yackov Berkun, Hadassah Mnt Scopus

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

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Yackov Berkun, Hadassah Mnt Scopus

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  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

  30. Berkun, JIA

  31. Rash Berkun, JIA

  32. Koebner Berkun, JIA

  33. Berkun, JIA

  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

  45. Berkun, JIA

  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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