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JIA and Other Rheumatic Diseases in Children

JIA and Other Rheumatic Diseases in Children. Norma Liburd , RN-BC , MN. Objectives. Define Juvenile Idiopathic Arthritis (JIA) and discuss the diagnostic criteria. Identify the subtypes of JIA and discuss characteristics of each.

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JIA and Other Rheumatic Diseases in Children

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  1. JIA and Other Rheumatic Diseases in Children Norma Liburd, RN-BC, MN

  2. Objectives • Define Juvenile Idiopathic Arthritis (JIA) and discuss the diagnostic criteria. • Identify the subtypes of JIA and discuss characteristics of each. • Name at least one NSAID, one biologic and one DMARD used in the treatment of JIA.

  3. A few more Objectives • Discuss three school related problems students with JIA have and intervention strategies for each. • Identify the criteria for classification of systemic lupus erythematosus. • Name the most common type of juvenile localized scleroderma. • Discuss the criteria for diagnosis of juvenile dermatomyositis, and treatment approaches

  4. Overview of JIA • New classification criteria proposed by the Pediatric Task Force of the International League of Associations for Rheumatology (ILAR) in 1997 • Chronic arthritis in childhood – one of the more frequent chronic illnesses of childhood. • An important cause of short and long-term disability

  5. Chronic arthritis in childhood: JIA • It’s not a single disease, but a group of related, genetically heterogeneous, phenotypically diverse immunoinflammatory disorders affecting joints and other structures, possibly activated by contact with an external antigen or antigens.

  6. JRA - Incidence/Prevalence • Published series are difficult to interpret due to classification, methodologies, heterogeneity • Incidence: (per year) 1/100,000 in Japan 20/100,000 in Norway • Prevalence: • 10 /100,000 in France • 400/100,000 in Australia • 113/ 100,000 • Arthritis Foundation: 300,000 children in the US have chronic arthritis. .

  7. JRA – Classification Criteria • JRA – American College of Rheumatology 1970 three types of onset: oligo (pauciarticular), polyarticular, & systemic in the first 6 months of onset • JCAJuvenile Chronic Arthritis (European League Against Rheumatism) 1977 • JIAJuvenile Idiopathic Arthritis proposed by the Pediatric Task force of the International League of Associations for Rheumatology ILAR (1993) – developed to achieve homogeneity within disease and categories.

  8. Sex Ratio • All types of JIA: • Girls: Boys 2:1 • Oligo JIA: • Girls: Boys 3:1 • JIA with uveitis • Girls: Boys 5-6:1 • Poly JRA: • Girls: Boys 3:1 • Systemic JRA: • Girls: Boys approx. 1:1

  9. JIA outcomes: Mortality Disease associated death rate is < 1% in Europe < 0.3% in North America These numbers represent a 4 Fold to 14 fold Increase in Mortality Rate Compared with General Population Causes are cardiac, infection & macrophage activation syndrome

  10. JRA outcome: functional abilities

  11. Age at onset <16 years Duration of Arthritis: 6 weeks Arthritis in one or more joints defined as swelling or effusion, or presence of two or more of the following signs: (in 1 or more joints) Limitation of ROM Tenderness or pain on motion Increased heat Exclusion of other diseases Classification Criteria for JIA

  12. Diagnostic Studies

  13. Diagnostic Tests • There is no lab test that diagnoses JIA The H&P should determine the labs, not the reverse • CBC • Rheumatoid factor • Antinuclear Antibody (ANA) – with titer • ESR or CRP • Anti-CCP (anti-cyclic citrullinated protein)

  14. Radiologic Studies X-rays Soft tissue swelling Osteoporosis Periosteal new bone formation Epiphyseal overgrowth Marginal erosions Narrowing of cartilaginous space Joint subluxation Bony fusion Dexascans Osteopenia Osteoporosis

  15. Immune mediated disease Abnormal immunoregulation Abnormal cytokine production in the inflammatory pathway (TNF, IL-6, IL-2R, IL-1alpha) Complex genetic predispositions HLA associations Environmental triggers Infections Trauma Stress Etiology

  16. Synovial lining is a thin membrane enclosing the joint space. The joint space contains fluid that bathes the joint and reduces friction on motion.

  17. With onset of inflammation, the synovial lining thickens and secretes more fluid, which may remain in the joint and cause swelling. The inflamed lining produces warmth, swelling, and pain. As inflammation progresses, the synovial lining grows over the cartilage and starts to erode it. As inflammation continues, changes include marked erosion of cartilage, cystic changes and thinning of the bone.

  18. Classification Criteria • 1. Systemic2.Oligoarthritis a. Persistentb. Extended 3.Polyarthritis (rheumatoid factor negative) 4.Polyarthritis (rheumatoid factor positive) 5. Psoriatic arthritis 6.Enthesitis-related arthritis 7. Undifferentiated arthritis a. Fits no other categoryb. Fits more than one categoryFrom Petty RE, Southwood TR, Baum J et al: Revision of the proposed classification criteria for juvenile idiopathic arthritis: Durban, 1997, J Rheumatol 25:199-1994, 1998.

  19. JIA Subtypes • Systemic Onset (5-15%) • Polyarticular Onset (20%) • Rheumatoid Factor Positive • Rheumatoid Factor Negative (85%) • Oligoarthritis (50-80%) • Juvenile psoriatic arthritis (7%) • Enthesitis related arthritis • Undifferentiated

  20. Systemic JIA • Definition: • Arthritis with, or preceded by, daily fever of at least 2 weeks’ duration • Fevers are quotidian (daily) for at least 3 days and is accompanied by one or more of the following: • Evanescent, non-fixed, erythematous rash • Generalized lymph node enlargement • Hepatomegaly and/or splenomegaly • Serositis

  21. Quotidian fever Intermittent fever of systemic JIA in a 3-year-old girl. The fever spikes usually occurred daily in the late evening to early morning (quotidian pattern), returned to normal or below normal, and were accompanied by severe malaise, tachycardia, and rash.

  22. Systemic JRA • Rash - • Salmon colored • Maculopapular – flat to slightly raised • Trunk and extremities • Migratory • Pruritic 5% • Fleeting • Persistent with fever spike

  23. Overview of Systemic JIA • 10-15% of all JRA patients • Broad peak of onset 1-5 years • M:F 1:1 • Variable number of joints • Il-6 is elevated and correlates with disease activity • Extraarticular symptoms: • Fever 100 % • Rash 95% • Hepatosplenomegaly, 85% • Lymphadenopathy 70% • Pericarditis 35% • Pleuritis 20%

  24. Macrophage Activation Syndrome • Rare devastating complication of systemic JIA. Etiology is uncertain. • Demonstration of macrophages ingesting other hematopoietic cells in marrow is diagnostic • Early recognition is life-saving • Looks somewhat like a flare up of systemic JRA but is different enough to allow for early recognition) • Associated with CMV, EBV, changes in meds • Mortality 10-20%

  25. Macrophage Activation Syndrome • Acute onset of fever with • Bruising, purpura, mucosal bleeding • Enlarged lymph nodes, liver, spleen • Elevated AST, ALT, PT, PTT, fibrin D-dimer • Elevated ferritin & triglycerides • Abrupt fall in WBC & platelets • Fall in ESR • Fall in fibrinogen, clotting factors • Often progresses to fatal DIC, hepatic failure, encephalopathy • Treatment: IV steroids, cyclosporin

  26. PolyarticularJIA - RF negative • Five or more joints in the first 6 months of disease • Asymmetric joint involvement • Large joints of knees, wrists, elbows and ankles often affected • Morning stiffness, joint pain • Intermittent low-grade fever

  27. Polyarticular- RF positive • Arthritis affecting 5 or more joints in the first 6 months of disease. • Similar to adult RA • Females with onset in adolescence • Rheumatoid nodules • Early onset of erosive synovitis • Symmetric joint involvement • Small joints of hands or feet are affected • TMJ: micronathia • Cervical spine may be affected

  28. Rheumatoid Nodules • Occur in 5-10% of children with JIA • Most frequently on elbow • Pressure points, digital flexor tendon sheaths, Achilles tendons, bridge of nose in child who wears glasses • Firm or hard, usually mobile, nontender. • Solitary or multiple, may change in size, may last months to years.

  29. Oligoarticular JIA • Arthritis in 1 to 4 joints during the first 6 months of disease • Girls 1 to 4 years • Knees, ankles, elbows • Painless swelling of joints is common • Uveitis: insidious, subacute • 15-20% have uveitis

  30. JIA: Oligo – persistent • No more than 4 joints affected throughout the disease course JIA: Oligo - extended • Affects a total of more than 4 joints after the first 6 months of disease. At least 1/3 of children with Oligoarticulararthritis fall into this categoryOutcome is more typical of RF+ polyarticular disease

  31. Uveitis in JIA • Intraocular inflammation affects iris and ciliary body • Usually insidious and may be asymptomatic • Activity of eye does not parallel joint disease • Slit lamp exam detects anterior chamber inflammation • Girls, ANA + and onset before age 7 at higher risk

  32. Prognosis of Uveitis in JIA • Very good in 25% of cases • 25% may require surgery for cataracts and/or glaucoma • 50% require prolonged treatment for moderate to severe chronic inflammation; however, the prognosis is generally good Complications: cataracts 20%, glaucoma 20%, band keratopathy 16% (end stage scarring)

  33. Uveitis in JIA • Usually occurs after onset of arthritis. Highest risk is within 2 years of onset of arthritis. Majority develop eye disease within 5-7 years after onset • 65% have bilateral involvement, unilateral may progress to bilateral • Treatment includes topical steroids, SQ Methotrexate, IV Remicade; SQ Humira and • Enbrel.

  34. OligoarticularANA+ <7 years at Dx Oligoarticular ANA+ 7 or older at Dx Oligoarticular ANA - <7 years at Dx Oligoarticular ANA – <7 years at Dx Systemic Q 3-4 months for 7 years, then yearly. Q 4-6 months for 7 yrs, then yearly. Q 4-6 months for 4 yrs, then yearly. Yearly. Slit Lamp Exam – JIA GuidelinesRheumatology & Ophthalmology sections of the American Academy of Pediatrics, 1993

  35. Guidelines for ophthalmological screening of children with JIA High risk – screen every 3 months Moderate risk – screen every 4-6 months Low risk: screen every 12 months All patients considered to be at low risk 7 yr after onset of arthritis; should have yearly ophthalmological exams indefinitely. All patients are considered to be at low risk 4 years after onset of arthritis, should have yearly ophthalmological exams indefinitely. All high risk patients are considered to be at medium risk 4 years after onset of arthritis. Modified from Yancy C, et.al, The Guidelines of the Rheumatology and ophthalmology sections of the AAP. Pediatrics 92:295-296, 2003.

  36. JIA: Psoriatic Arthritis • Arthritis and psoriasis or • Arthritis with 2 of the following: • Dactylitis - sausage like swelling of toe or finger • Nail pitting • Psoriasis in a first degree relative (parents, siblings) • Slightly more females • Symmetrical involving large and small joints

  37. JRA: SpondyloarthropathyJIA:Enthesitis related arthritis • Arthritis and enthesitis • Arthritis or enthesitis with at least 2 of the following: • Sacroiliac joint tenderness and/or inflammatory lumbosacral pain • Presence of HLA-B27 • Onset of arthritis in a male after age 6 years • Ankylosingspondylitis, Enthesitis Related Arthritis, Sacroiliitis with inflammatory bowel disease, Reiter’s syndrome or acute anterior uveitis in a first-degree relative.

  38. JRA: SpondyloarthropathyJIA:Enthesitis related arthritis • Primarily affects boys 8 years and older • Affects large joints of lower extremities • Heel pain and Achilles tendonitis • Sacroiliitis (90% of cases) • Iritis (20% of cases) generally acute process • Low grade fevers • Decreased appetite

  39. Medications • NSAIDs • DMARDs: Methotrexate, Plaquenil, Sulfasalazine • Biologic response modifiers • Glucocorticosteroids • Miscellaneous

  40. NSAIDS • FDA approved for pediatric use • Aspirin • Tolmetin • Naproxen • Ibuprofen • Indomethacin • Meloxicam (Mobic) • Celebrex

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