A Case of Idiopathic Juvenile Arthritis


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A Case of Idiopathic Juvenile Arthritis

  2. 2. history <ul><li>14 year boy presented with fever of 1 month duration. </li></ul><ul><li>fever low grade, with no rigors, more in the evenings. </li></ul><ul><li>No h/o cough, expectorations, dyspnea, chest pain, palpitations. </li></ul><ul><li>No h/o weight loss, night sweats. </li></ul><ul><li>No h/o dysuria, hematuria, pain abdomen </li></ul>
  3. 3. <ul><li>No h/o loose stools, abdominal pain, constipation, weight loss. </li></ul><ul><li>h/o myalgia, joint pains. </li></ul><ul><li>No h/o rashes, bleeding from gums, or any other mucosal bleeds. </li></ul><ul><li>No h/o seizures, altered sensorium. </li></ul><ul><li>No h/o any skin lesions. </li></ul><ul><li>Past history: </li></ul><ul><li>No similar illness. </li></ul><ul><li>No h/o transfusions, tattooing. </li></ul><ul><li>Patient was treated by a private practitioner, but fever tend to recur. </li></ul>
  4. 4. <ul><li>While in hospital the patient complained of severe pain in the knee and left ankle pain. </li></ul><ul><li>The patient gave h/o pain and swelling of joints on & off for past 6 months for which he was treated with analgesics – self medication. The pain disappear but to re appear later. </li></ul>
  5. 5. <ul><li>Family history: </li></ul><ul><li>No h/o PT,BA, DM, SHT, psoriasis </li></ul><ul><li>Personal history: </li></ul><ul><li>Nil significant </li></ul>
  6. 6. On examination <ul><li>Pt conscious, oriented </li></ul><ul><li>Febrile, temp- 100 F. </li></ul><ul><li>pallor+ </li></ul><ul><li>No icterus, cyanosis, clubbing, pedal edema, LNE. </li></ul><ul><li>Pulse- 82/min , regular. </li></ul><ul><li>BP- 110/70 mm hg. </li></ul>
  7. 7. <ul><li>Cvs- s1, s2 heard, no murmurs. </li></ul><ul><li>RS- nvbs heard, no added sounds </li></ul><ul><li>p/a- soft, non tender, </li></ul><ul><li>Spleen palpable- 2 cm below left costal margin, soft, non tender. </li></ul><ul><li>Liver not palpable, </li></ul><ul><li>No free fluid. </li></ul><ul><li>Cns- no focal deficits </li></ul>
  8. 8. <ul><li>On examination of joints: </li></ul><ul><li>The left ankle , left knee , PIP join of right middle finger joints swollen. </li></ul><ul><li>Warmth + </li></ul><ul><li>Tenderness in the joint line+ </li></ul><ul><li>Movements restricted due to pain in the above joints. </li></ul><ul><li>Other joints appeared normal </li></ul>
  9. 9. <ul><li>Summary: </li></ul><ul><li>14 year old boy with </li></ul><ul><li>Low grade fever </li></ul><ul><li>Anemia </li></ul><ul><li>Splenomegaly </li></ul><ul><li>Poly arthritis </li></ul>
  11. 14. Diferential diagnosis <ul><li>Septic arthritis </li></ul><ul><li>Tuberculosis </li></ul><ul><li>Hemarthrosis. </li></ul><ul><li>Juvenile idiopathic arthritis </li></ul><ul><li>SLE </li></ul><ul><li>Trauma. </li></ul><ul><li>Psoriatic arthritis. </li></ul><ul><li>Enteropathic arthritis-IBD </li></ul>
  12. 15. Treatment given <ul><li>Iv fluids </li></ul><ul><li>Cetriaxone- iv </li></ul><ul><li>Tab.Artemether- lumefantrine. </li></ul><ul><li>Paracetamol. </li></ul><ul><li>NSAID- Indomethacin </li></ul>
  13. 16. INVESTIGATIONS <ul><li>CBC: </li></ul><ul><li>Hb- 9 grams </li></ul><ul><li>TC-4600 cells/cumm </li></ul><ul><li>DC- N-55,L-45, </li></ul><ul><li>ESR- 25/45 </li></ul><ul><li>Platelets- 1.5 lakh </li></ul><ul><li>Rbc-4 million. </li></ul><ul><li>Rft: </li></ul><ul><li>Urea- 35 mg </li></ul><ul><li>Creatinine- 1.0 mg </li></ul>
  14. 17. <ul><li>Smear for mp/mf- negative </li></ul><ul><li>Widal – negative study </li></ul><ul><li>Mantoux- negative </li></ul><ul><li>Chest xray- normal. </li></ul><ul><li>MSAT for leptospirosis- negative </li></ul><ul><li>Peripheral smear- normocytic normochromic anemia, no immature cells seen. </li></ul><ul><li>Bone marrow- normal study, no blasts seen. </li></ul><ul><li>PT, APTT- Normal. </li></ul>
  15. 18. <ul><li>HIV – negative </li></ul><ul><li>HBS Ag- negative </li></ul><ul><li>anti HCV- negative </li></ul><ul><li>Stool examination- occult blood negative. </li></ul><ul><li>Urine culture- no organism grown </li></ul><ul><li>Blood culture- no organism grown. </li></ul><ul><li>RA factor- negative. </li></ul><ul><li>ANA- positive </li></ul><ul><li>Anti DS DNA- negative </li></ul>
  16. 20. <ul><li>Repeat CBC- </li></ul><ul><li>TC-5100 cells </li></ul><ul><li>DC- N- 52, L-45. </li></ul><ul><li>ESR-30/55. </li></ul><ul><li>Aspiration from joint – serous fluid+, no pus. </li></ul><ul><li>CRP – positive. </li></ul><ul><li>Anti CCP –POSITIVE. </li></ul><ul><li>Serum uric acid- 4.5 mg/dl </li></ul>
  17. 21. <ul><li>IN view of </li></ul><ul><li>Age < than 16 years </li></ul><ul><li>Arthritis, 3 joints involved, </li></ul><ul><li>Duration of symptoms> than 6 months. </li></ul><ul><li>Low grade fever, anemia, spleno megaly, </li></ul><ul><li>ANA , anti CCP , CRP postivity </li></ul>
  18. 22. <ul><li>Final diagnosis </li></ul><ul><li>JUVENILE IDIOPATHIC ARTHRITIS. </li></ul>
  19. 23. <ul><li>Juvenile idiopathic arthritis </li></ul>
  20. 24. JUVENILE IDIOPATHIC ARTHRITIS American College of Rheumatology Revised Criteria <ul><li>age of onset < 16 years </li></ul><ul><li>arthritis of one or more joints </li></ul><ul><li>duration of disease > 6 weeks </li></ul><ul><li>other conditions which present with arthritis in childhood must be excluded </li></ul>
  21. 25. JUVENILE IDIOPATHIC ARTHRITIS Epidemiology <ul><li>described in all races and geographic areas </li></ul><ul><li>incidence: 6 – 19.6 cases/100,000 children </li></ul><ul><li>prevalence: 16-150/100,000 </li></ul><ul><li>females predominate 2:1 </li></ul>
  22. 27. JUVENILE RHEUMATOID ARTHRITIS Etiology and Pathogenesis <ul><li>unknown </li></ul><ul><li>combination of factors </li></ul><ul><ul><li>environment (infection, trauma, stress) </li></ul></ul><ul><ul><li>autoimmunity </li></ul></ul><ul><ul><li>immunogenetic </li></ul></ul>
  23. 28. PATHOGENESIS <ul><li>The etiology of JRA is unknown </li></ul><ul><li>Chronic inflammation of synovium is characterized by B lymphocyte infiltration and expansion. Macrophages and T-cell invasion are associated with the release of </li></ul><ul><li>cytokines, which evoke synoviocyte proliferation. </li></ul>
  24. 29. PATHOGENESIS <ul><li>Studies have suggested exaggerated immune reactivity of several types of cells in predisposed children, may be in response to certain viral infections </li></ul><ul><li>There is recruitment of T cells for antigens in the joint synovium </li></ul><ul><li>This is made possible by certain HLA types DR4 (poly) DR8 and DR5 (pauci) </li></ul>
  25. 30. PATHOGENESIS <ul><li>In many patients, predominance of cytokines associated with tissue destruction, including interleukin-6 and TNF, suggests the possibility of improved responsiveness to specific biologic agents targeting these factors. </li></ul><ul><li>A 2001 study by Scola et al found synovium to contain messenger RNA for vascular endothelial growth factor, angiopoietin 1, and their respective receptors, suggesting that induction of angiogenesis by products of lymphocytic infiltration may be involved in persistence of disease </li></ul>
  26. 31. EXAMINATION <ul><li>A detailed physical examination is a critical tool in diagnosing JRA. </li></ul><ul><li>Physical findings are important to provide criteria for diagnosis and to detect abnormalities suggestive of other possible diagnoses </li></ul><ul><li>Arthritis on examination is defined as either joint swelling (although trauma can also cause swelling and may need to be excluded) or the combination of limited motion with pain (on motion or to palpation). </li></ul>
  27. 32. <ul><li>The hips and small joints in the spine, when affected by synovitis, do not demonstrate swelling but demonstrate the combination of loss of motion and pain </li></ul><ul><li>In individuals with pauciarticular JRA, 4 or fewer joints are affected; often, only a single joint is affected. </li></ul><ul><li>Typically, large weight-bearing joints, knees, and ankles are affected. </li></ul>
  28. 33. <ul><li>In persons with polyarticular JRA, 5 or more joints are affected. </li></ul><ul><li>Weight-bearing joints are affected, and symmetric involvement of small joints in the hands is found </li></ul><ul><li>Pain with decreased range of motion in the cervical spine is sometimes found. </li></ul><ul><li>Involvement of a few small joints in the hands is atypical and suggests eventual development of polyarticular JRA </li></ul>
  29. 34. JUVENILE RHEUMATOID ARTHRITIS Onset Types <ul><li>pauciarticular ( < 4 joints) </li></ul><ul><li>polyarticular ( > 5 joints) </li></ul><ul><li>systemic (arthritis with fever and rash) </li></ul>
  30. 35. ILAR Proposed Classification Criteria <ul><li>Juvenile Idiopathic Arthritis (JIA) </li></ul><ul><li>systemic </li></ul><ul><li>polyarticular RF+ </li></ul><ul><li>polyarticular RF- </li></ul><ul><li>oligoarticular </li></ul><ul><ul><li>persistent </li></ul></ul><ul><ul><li>extended </li></ul></ul><ul><li>psoriatic arthritis </li></ul><ul><li>enthesitis-related arthritis </li></ul><ul><li>undifferentiated arthritis </li></ul>
  31. 36. JUVENILE RHEUMATOID ARTHRITIS Clinical features: pauciarticular disease <ul><li>40-60% of patients with JRA. </li></ul><ul><li>Preferentially affect girls. </li></ul><ul><li>insidious onset </li></ul><ul><li>morning irritability/stiffness subtle </li></ul><ul><li>systemic symptoms: usually absent </li></ul><ul><li>large joints (rarely hip), asymmetric involvement. </li></ul><ul><li>Strong association between HLA A2. HLA DRB1. </li></ul><ul><li>M/C- knees and ankles. </li></ul><ul><li>Uveitis- m/c extra articular manifestation. </li></ul>
  32. 37. JUVENILE RHEUMATOID ARTHRITIS Laboratory Studies: Pauciarticular Disease <ul><li>CBC: normal </li></ul><ul><li>ESR: usually normal </li></ul><ul><li>ANA: frequently positive </li></ul><ul><li>RF: usually negative </li></ul><ul><li>synovial fluid: inflammatory </li></ul><ul><li>x-ray findings: soft tissue swelling, periarticular osteoporosis, growth disturbance, loss of joint space </li></ul>
  33. 41. JUVENILE RHEUMATOID ARTHRITIS Clinical features: polyarticular disease <ul><li>30-40% of patients with JRA </li></ul><ul><li>morning irritability/stiffness more prominent </li></ul><ul><li>systemic symptoms: mild to moderate </li></ul><ul><li>large and small joints including cervical spine, symmetric involvement </li></ul><ul><li>uveitis 5% </li></ul><ul><li>subtypes </li></ul>
  34. 42. JUVENILE RHEUMATOID ARTHRITIS Laboratory features: polyarticular disease <ul><li>WBC  , Hb  , platelets WNL to  </li></ul><ul><li>ESR  to   </li></ul><ul><li>ANA may be positive </li></ul><ul><li>RF may be positive </li></ul><ul><li>Synovial fluid: inflammatory </li></ul><ul><li>X-ray findings: soft tissue swelling, periarticular osteoporosis, joint space narrowing, erosions </li></ul>
  35. 44. JUVENILE RHEUMATOID ARTHRITIS Clinical features: systemic disease <ul><li>10-20% of patients with JRA </li></ul><ul><li>prominent systemic symptoms: fever, rash, lymphadenopathy, hepatosplenomegaly, pericarditis, pleuritis </li></ul><ul><li>arthritis may be absent for months to years </li></ul><ul><li>uveitis uncommon </li></ul>
  36. 46. JUVENILE RHEUMATOID ARTHRITIS Laboratory studies: systemic disease <ul><li>WBC   , Hgb  , platelets  to   , </li></ul><ul><li>ESR   to    </li></ul><ul><li>ANA and RF usually negative </li></ul><ul><li>x-rays : soft tissue swelling </li></ul>
  37. 47. JUVENILE RHEUMATOID ARTHRITIS Extra-articular Manifestations <ul><li>generalized or local growth disturbances </li></ul><ul><li>delayed puberty </li></ul><ul><li>pericarditis, myocarditis, rarely endocarditis </li></ul><ul><li>pleural effusion, </li></ul><ul><li>pneumonitis, pulmonary fibrosis- CAPLAN’s syndrome </li></ul><ul><li>hepatitis </li></ul><ul><li>Renal involvement is rare </li></ul>
  38. 48. JUVENILE RHEUMATOID ARTHRITIS Treatment <ul><li>supportive not curative </li></ul><ul><li>involves multidisciplinary team approach </li></ul><ul><li>goals: </li></ul><ul><ul><li>to suppress articular and/or systemic inflammation with as little risk as possible </li></ul></ul><ul><ul><li>to maintain function/prevent disabilities </li></ul></ul><ul><ul><li>to foster normal psychological and social development </li></ul></ul><ul><li>heterogenity of disease mandates individualization </li></ul>
  39. 49. JUVENILE RHEUMATOID ARTHRITIS Treatment: physical measures <ul><li>heat: analgesia </li></ul><ul><li>muscle relaxation </li></ul><ul><li>splinting: provide joint rest </li></ul><ul><li>maintain functional position </li></ul><ul><li>correct deformities </li></ul><ul><li>exercise: passive, active assisted and </li></ul><ul><li>active range of motion </li></ul><ul><li>general conditioning </li></ul><ul><li>rest </li></ul>
  40. 50. Pharmacologic Therapy <ul><li>NSAIDs </li></ul><ul><li>Glucocorticoids </li></ul><ul><li>DMARD </li></ul><ul><li>Anticytokine therapy. </li></ul><ul><li>Chemo theraputic agents. </li></ul><ul><li>Autologous stem cell transplant in severe systemic JIA. </li></ul>
  41. 51. NSAID <ul><li>Naproxen, ibuprofen, diclofenac, and indomethacin. </li></ul><ul><li>Aspirin is not the drug of first choice because of the increased frequency of gastric toxicity and hepatotoxicity when compared to other NSAID medications </li></ul><ul><li>Predicting which individual patient will respond to a particular NSAID is not possible; sometimes, after 1-2 months of treatment, those persons who do not respond may benefit from changing to a different NSAID. </li></ul>
  42. 52. NSAIDs <ul><li>Pros: </li></ul><ul><ul><li>Analgesic, Antipyretic, Anti-inflammatory </li></ul></ul><ul><li>Cons: </li></ul><ul><ul><li>Don’t alter disease progression </li></ul></ul><ul><ul><li>Ineffective in Erosive disease </li></ul></ul><ul><li>GI/Ulcers </li></ul><ul><li>Hepatotoxicity </li></ul><ul><li>Nephrotoxicity </li></ul><ul><li>AIN </li></ul><ul><li>Bleeding – antiplatelet </li></ul><ul><li>Rash </li></ul><ul><li>Aseptic meningitis </li></ul>
  43. 53. <ul><li>DMARD: </li></ul><ul><li>GOLD salts </li></ul><ul><li>D- Pencillamine </li></ul><ul><li>sulfasalazine </li></ul>
  44. 54. Methotrexate <ul><li>may affect immune function </li></ul><ul><li>Dose10-25 mg/m2/wk PO/IM/SC as a single dose or divided into 2 doses qwk; many pediatric rheumatologists increase dose (not to exceed 30 mg/m2, approximately equivalent to 1 mg/kg); administer with folic acid 1-2 mg PO qd or folinic acid 2.5-5 mg PO qwk </li></ul>
  45. 55. Methotrexate <ul><li>Dihydrofolate reductase inhibitor </li></ul><ul><ul><li>Well tolerated, Mono/Combo </li></ul></ul><ul><ul><li>Onset: 6-12 weeks </li></ul></ul><ul><li>Metabolism: Liver </li></ul><ul><li>Clearance: Kidneys </li></ul><ul><li>Monitoring: </li></ul><ul><ul><li>Baseline:CXR, PFTs, HIV, HBV/HCV </li></ul></ul><ul><ul><li>CBC, LFTs Q4-8 weeks </li></ul></ul><ul><ul><li>Caution with CRI </li></ul></ul><ul><li>Nausea </li></ul><ul><li>Mucosal ulcerations </li></ul><ul><li>Fatigue & Flu-like symptoms </li></ul><ul><li>BM Toxicity </li></ul><ul><li>Hepatotoxicity </li></ul><ul><li>Treat with Folic acid, 1 mg/d </li></ul>
  46. 56. Corticosteroids <ul><li>Decrease cytokines </li></ul><ul><li>Slow Joint Inflammation. </li></ul><ul><li>Intra lesional triamcinalone hexaceetonide </li></ul><ul><li>Insomnia </li></ul><ul><li>Emotional lability </li></ul><ul><li>Fluid retention </li></ul><ul><li>Weight gain </li></ul><ul><li>HTN </li></ul><ul><li>Hyperglycemia </li></ul><ul><li>Osteoporosis </li></ul><ul><ul><li>Bisphosphonates: >5mg/d for >3months </li></ul></ul><ul><li>Cataracts </li></ul><ul><li>Avascular necrosis </li></ul><ul><li>Myopathy </li></ul><ul><li>Psychosis </li></ul>
  47. 57. Leflunomide <ul><li>Inhibits dihydrooratate dehydrogenase </li></ul><ul><ul><li>Dec. activated T-cells </li></ul></ul><ul><ul><li>Onset: rapid </li></ul></ul><ul><ul><li>Efficacy: ≤6 weeks </li></ul></ul><ul><li>Monitoring: </li></ul><ul><ul><li>CBC, LFTs </li></ul></ul><ul><li>Derm - rash, alopecia </li></ul><ul><li>Diarrhea </li></ul><ul><li>BM toxicity </li></ul><ul><li>Hepatotoxicity </li></ul>
  48. 58. Azathioprine <ul><li>Corticosteroid-sparing </li></ul><ul><li>Monitoring: </li></ul><ul><ul><li>CBC Q1-2 months </li></ul></ul><ul><ul><li>AST/ALT </li></ul></ul><ul><li>Infection </li></ul><ul><li>BM Toxicity </li></ul><ul><li>Hepatitis </li></ul><ul><li>Malignancy </li></ul>
  49. 59. Cyclophosphamide <ul><li>Alkylating agent </li></ul><ul><li>Monitoring: </li></ul><ul><ul><li>CBC, UA monthly </li></ul></ul><ul><ul><li>Yearly UA +/- Cytology </li></ul></ul><ul><li>Alopecia </li></ul><ul><li>Nausea </li></ul><ul><li>Infection </li></ul><ul><li>BM suppression  pancytopenia </li></ul><ul><li>Infertility – pretreat women with Leuprolide </li></ul><ul><li>Renal: hemorrhagic cystitis, bladder malignancy – treat with mesna. </li></ul>
  50. 60. Anticytokine therapy <ul><li>Anti-TNF alpha agents </li></ul><ul><ul><li>Etanercept </li></ul></ul><ul><ul><li>Infliximab </li></ul></ul><ul><ul><li>Adalimumab </li></ul></ul><ul><ul><li>IL-1 receptor antagonist (Anakinra). </li></ul></ul><ul><ul><li>Abatacept- CTLA-4 IG </li></ul></ul>
  51. 61. TNF-a Inhibitors <ul><li>Anti-inflammatory </li></ul><ul><li>Block TNF-α (proinflammatory cytokine) </li></ul><ul><li>Etanercept, Adalimumab (SQ), Infliximab (IV) </li></ul><ul><li>Combo therapy with MTX </li></ul><ul><li>Injection site reaction </li></ul><ul><li>Infection </li></ul><ul><li>Reactivated TB </li></ul><ul><li>Infliximab </li></ul><ul><ul><ul><li>infusion reaction </li></ul></ul></ul><ul><li>Pancytopenia </li></ul><ul><li>Autoantibody/SLE-like </li></ul><ul><li>Exacerbate CHF </li></ul><ul><li>Malignancy – lymphoma </li></ul>
  52. 62. Etanercept <ul><li>Acts by binding and inhibiting TNF, a cytokine that contributes to inflammatory and immune response. </li></ul><ul><li>Contraindications Documented hypersensitivity; sepsis </li></ul><ul><li>Interactions -Do not administer within 3 months of live virus vaccines </li></ul><ul><li>Precautions Caution in impaired renal function and asthma; discontinue administration if a serious infection develops; adverse effects may include injection site pain, localized erythema, rash, URI symptomatology, GI upset, nausea, vomiting, rhinitis, and cough </li></ul>
  53. 63. Disease Course <ul><li>Long Remission </li></ul><ul><ul><li>10% </li></ul></ul><ul><li>Intermittent Disease </li></ul><ul><ul><li>15-30% </li></ul></ul><ul><li>Progressive Disease </li></ul>
  54. 64. JUVENILE RHEUMATOID ARTHRITIS Prognosis <ul><li>chronic disease which cannot be cured </li></ul><ul><li>characterized by flares and remissions </li></ul><ul><li>after 10 years or more: </li></ul><ul><ul><li>31%-55% persistent active disease </li></ul></ul><ul><ul><li>31% (9% - 48%) Steinbrocker Class III and IV </li></ul></ul>
  55. 65. JUVENILE RHEUMATOID ARTHRITIS Poor Prognostic Signs <ul><li>pauciarticular </li></ul><ul><ul><li>long duration of active disease </li></ul></ul><ul><ul><li>conversion to polyarticular disease (30%) </li></ul></ul><ul><ul><li>chronic uveitis </li></ul></ul><ul><li>polyarticular </li></ul><ul><ul><li>long duration of active disease </li></ul></ul><ul><ul><li>articular erosions </li></ul></ul><ul><ul><li>RF positivity/rheumatoid nodules </li></ul></ul><ul><li>systemic </li></ul><ul><ul><li>conversion to polyarticular disease (25-50%) </li></ul></ul>
  56. 66. <ul><li>References: </li></ul><ul><li>KELLY’S RHEUMATOLOGY. </li></ul><ul><li>HARRISONS. </li></ul>
  57. 67. <ul><li>Thank you </li></ul>