1. DR.IBTISAM JALI MEDICAL DEMONSTRATOR
JRA
DR.IBTISAM JALI
MEDICAL DEMONSTRATOR

2. Systemic Pauciarticular Polyarticular
Systemic
Pauciarticular
Polyarticular
Percent of JRA patients
10-15 50
30-40
Sex
F = M
F > M
Age
any <17 years
peak 2-3 years, rare >10
peaks 2-5, years
Joints
any
large joints, but rarely hips
any, rare to start in hip
Fever, rash, lymphadenopathy, hepatosplenomegaly
yes no
Uveitis
rare
20 percent, esp ANA +
less frequent
Laboratory abnormalities
Leukocytosis
marked
Anemia
mild
Elevated ESR
ANA
absent
low titer common
low titer common in younger
Rheumatoid factor
10-20 percent in those >10 years
Destructive arthritis
>50 percent
Disease modifying drug
commonly used
rarely used

3. PAUCIARTICULAR JRA
True pauciarticular onset JRA is usually responsive to (NSAIDs).
Methotrexate and other immunosuppressive drugs are rarely, if ever, required.
patients does not respond to NSAIDs or intra-articular steroid injections, may benefit from methotrexate or anti TNF (eg, etanercept or adalimumab).
(extended pauciarticular subset) additional joint involvement after an initial pauciarticular onset low-dose oral methotrexate may be effective

4. POLYARTICULAR JRA
Initial therapy with NSAIDs, If there is no response to the initial NSAID by three weeks, the first NSAID is discontinued and a second NSAID is started.
If the patient continues to be unresponsive to NSAID therapy alone, a second line drug is recommended; methotrexate at a dose of 10mg/m2 BSA/week or anti-TNF.
Corticosteroids; Systemic, Injection 
Leflunomide not FDA approved for pediatric use
Sulfasalazine has been shown to be beneficial for many children with polyarticular JRA Sulfasalazine does not prevent chronic changes and therefore should not be relied upon in erosive disease
adalimumab approved by the FDA for the treatment of moderate to severe JRA
Infliximab (Remicade®), a chimeric mouse-human monoclonal antibody with affinity for TNF-alpha. It is not approved by the FDA for treatment of JRA.
Abatacept (Orencia) has recently been approved by the United States FDA for the treatment of JRA

5. SYSTEMIC JRA
Nonsteroidal anti-inflammatory drugs (NSAIDs) alone are effective for many children with systemic onset JRA.
If NSAIDs are ineffective, second line agents such as corticosteroids, or methotrexate may be considered.
For those with the most difficult disease which is refractory to conventional therapy, biologic agents are used.
Infliximab and adalimumab, monoclonal antibodies to TNF alpha also have been used with varying success in children with systemic JRA
biological agents that target IL-1 and IL-6 activity, such as anakinra and thalidomide, human recombinant anti-interleukin-6 monoclonal antibody; MRA, tocilizumab, atlizumab
Cyclosporine has received increased attention over the past few years in systemic onset JRA. apparent usefulness in the treatment of DIC/macrophage activation syndrome
bone marrow transplantation should be restricted to only the most severely affected children

6. Macrophage activation syndrome
Macrophage activation syndrome — severe and life-threatening complication of systemic JRA,
typically occurs within the first few days or weeks following the initiation of therapy with aspirin, other nonsteroidal antiinflammatory drugs, sulfasalazine, or gold salts.
may follow viral and bacterial infections, or
occur without any evident initiating event.
It is thought to be caused by the activation of T lymphocytes and macrophages, resulting in the uncontrolled release of inflammatory cytokines ("cytokine storm").
Children may present with spontaneous bleeding, bruising, or shock. The hemoglobin, platelet count, and serum fibrinogen typically drop precipitously secondary to consumptive coagulopathy. Fibrin split products in the peripheral circulation are generally the earliest confirmatory sign of DIC.
Treatment consists of fresh frozen plasma, corticosteroids, and supportive care. All NSAIDs and other antirheumatic medications should be withdrawn since they may exacerbate the illness except corticosteroids and cyclosporine.

7. THANKS