Juvenile Idiopathic Arthritis

JIA, JRA, Pediatric rheumatic disease, juvenile rheumatoid arthritis

1. Juvenile Idiopathic Arthritis (JIA) Dr. Virendra Kumar Gupta MD Pediatrics,MIAP Fellowship In pediatric Gastroentero-Hepatology & Liver Transplantation Assistant Professor Institute of Paediatric Gastroenterology Nims University Jaipur  

2. 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. Also named- Juvenile Rheumatoid Arthritis (JRA) Juvenile chronic Arthritis(JCA) JUVENILE IDIOPATHIC ARTHRITIS (JIA)

3. unknown combination of factors environment (infection, trauma, stress) autoimmunity immunogenetic Etiology and Pathogenesis

4. Arthritis Chronic synovial inflammation leading to bone/joint erosion Morning stiffness, limp, or falling often Easy fatigability Joint swelling Minimal pain Joint NEVER red or exquisitely tender Alteration of activities Loss of function CLINICAL MANIFASTATION

5. Age: < 16y at time of onset Duration: at least 6 weeks Arthritis in one or more joints Exclusion of other rheumatologic d/o Subgroup named after 6 months Pauciarticular ( Oligoarticular ): 4 or fewer joints Polyarticular : 5 or more joints Systemic: arthritis with fever JUVENILE IDIOPATHIC ARTHRITIS American College of Rheumatology Revised Criteria

6. Oligoarticular Persistent Extended (>4 joints after 6 months) RF Positive Polyarticular RF Negative Polyarticular Systemic Onset Psoriatic Arthritis Enthesitis -related Arthritis Other classification system

7. Juvenile Idiopathic Arthritis (JIA) 1-oligoarticular persistent extended 2-polyarticular RF+ 3-polyarticular RF- 4-systemic 5-psoriatic arthritis 6-enthesitis-related arthritis 7-undifferentiated arthritis ILAR Proposed Classification Criteria

8. Pauci JRA 4 or fewer joints Large joints: knees, ankles, wrists NOT HIP Serology Positive ANA Negative RF Main morbidity ASYMPTOMATIC ANTERIOR UVEITIS (assoicated with positive ANA) Can lead to blindness

9. Poly JRA 5 or more involved joints Small and large joints PIP, MCP, wrist Rheumatoid nodules ANA may be positive RF may be + or – If + then worse prognosis

10. Systemic JRA Males = Females Quotidian fever Arthritis Visceral involvement HSM LAD Serositis Leukocytosis Rash Evanescent, salmon colored ANA and RF negative

11. “ Fleeting salmon-color rash ” Macular or wheal-like Not pruritic Irregular May coalesce with fever

12. Enthesitis-related JIA Enthesis: insertion of ligaments and tendons into bone Asymmetrical arthritis affected 4 or fewer joints Male predominance Spondyloarthritis

13. Enthesitis of axial skeleton and sacroiliac joints. Present with back pain Loss of lumbosacral mobility Oligoarthritis of joints of lower extremities Common presentation Male with back pain, morning stiffness that is relieved w/ exercise Labs HLA-B27 positive Increased ESR ANA and RF are NEGATIVE Radiology Bamboo Spine Treatment NSAIDS, Sulfasalazine, Mtx Ankylosing spondylitis

14. generalized or local growth disturbances delayed puberty pericarditis, myocarditis, rarely endocarditis pleural effusion, pneumonitis, pulmonary fibrosis- CAPLAN’s syndrome hepatitis Renal involvement is rare JUVENILE RHEUMATOID ARTHRITIS Extra-articular Manifestations

15. supportive not curative involves multidisciplinary team approach goals: to suppress articular and/or systemic inflammation with as little risk as possible to maintain function/prevent disabilities to foster normal psychological and social development heterogenity of disease mandates individualization JUVENILE RHEUMATOID ARTHRITIS Treatment

16. heat: analgesia muscle relaxation splinting: provide joint rest maintain functional position correct deformities exercise: passive, active assisted and active range of motion general conditioning rest JUVENILE RHEUMATOID ARTHRITIS Treatment: physical measures

17. NSAID’S Glucocorticoids DMARD Anticytokine therapy. Chemo theraputic agents. Autologous stem cell transplant in severe systemic JIA. Pharmacologic Therapy

18. Pros: Analgesic, Antipyretic, Anti-inflammatory Cons: Don’t alter disease progression Ineffective in Erosive disease NSAIDs GI/Ulcers Hepatotoxicity Nephrotoxicity AIN Bleeding – antiplatelet Rash Aseptic meningitis

19. Nonsteroidal anti-inflammatory drugs (NSAIDs) Medications Doses (mg / kg) Side effects Aspirin 50-120 Stomack pain, vomiting, gastrointestinal bleedings, headache, blood in the urine, fluid retention, thinning and scarring of the skin (especially with naproxen), stomach ulcer (aspirin) . Ibuprofen 10-30 Tolmentin 10-15 Naproxen 5-20

20. Slow-acting anti-rheumatic drugs (SAARDs) Medications Doses (mg / kg) Side effects Hydroxychlo-roquine (Plaquenil) 5-7 Upset stomach, skin rash and a eye damage. A child who takes this drug should have his/her eyes examined at least every six months by an ophthalmologist Sulfasalazine (Azulfadine)

21. Gold compaunds Medications Doses(weekly, depending from body weight ) Side effects Auranofin, Ridaura, Myochrysine Solganol 20 kg – 10 mg 30 kg – 20 mg 40 kg – 30 mg 50 kg – 40 mg > 50 kg – 50 mg Skin rash, mouth sores, kidney problems, a low blood count or anemia

22. Slow-acting anti-rheumatic drugs (SAARDs) Medications Week of treatment Doses (mg / kg) Side effects Penicillamine ( DePen , Cuprimine ) 0-2 2-4 4-6 6-8 10-14 25-50 50-100 100 100-150 150-200 Diarrhea, skin rash, low blood counts, nausea or vomiting, stomach pain, loss of appetite, swollen glands, unusual bleeding or bruising

23. Immune System Medications ( Cytostatics) Medications Doses Side effects Methotrexate (Rbeumatrex) Azathioprine (Imuran) Cyclophosphamide (Cytoxan) Typically 7.5 to 25 mg a week Loss of appetite, nausea or vomiting, skin rash, unusual bleeding or bruising, tiredness or weakness, sterility.

24. Biologic Agents, which blocks the protein TNF Etanercept (Enbrel) Infliximab (Remicade) Glucocorticoid Drugs (Dexamethasone, Methylprednisolone, Cortef, Prednisolone and Prednisone) Analgesics (acetaminophen [Tylenol, Panadol], tramadol [Ultram]) Other medications

25. Therapeutic exercises Sports and Recreational Activities Splints Very important parts of treatment for juvenile arthritis:

26. Morning Stiffness Relief Diet Eye Care Dental Care Surgery Very important parts of treatment for juvenile arthritis:

27. Disease Course Long Remission 10% Intermittent Disease 15-30% Progressive Disease

28. JUVENILE RHEUMATOID ARTHRITIS Prognosis chronic disease which cannot be cured characterized by flares and remissions after 10 years or more: 31%-55% persistent active disease 31% (9% - 48%) Steinbrocker Class III and IV

29. JUVENILE RHEUMATOID ARTHRITIS Poor Prognostic Signs pauciarticular long duration of active disease conversion to polyarticular disease (30%) chronic uveitis polyarticular long duration of active disease articular erosions RF positivity/rheumatoid nodules systemic conversion to polyarticular disease (25-50%)

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