Transcript JRA
JRA
DR.IBTISAM JALI
MEDICAL DEMONSTRATOR
Systemic
Pauciarticular
Polyarticular
Percent of JRA patients
10-15
50
30-40
Sex
F=M
F>M
F>M
Age
any <17 years
peak 2-3 years, rare
>10
peaks 2-5, 10-14 years
Joints
any
large joints, but rarely
hips
any, rare to start in hip
Fever, rash,
lymphadenopathy,
hepatosplenomegaly
yes
no
no
Uveitis
rare
20 percent, esp ANA +
less frequent
Leukocytosis
marked
no
no
Anemia
marked
no
mild
Elevated ESR
marked
mild
mild
ANA
absent
low titer common
low titer common in
younger
Rheumatoid factor
rare
absent
10-20 percent in those
>10 years
Destructive arthritis
>50 percent
rare
>50 percent
Disease modifying drug
commonly used
rarely used
commonly used
Laboratory abnormalities
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 lowdose oral methotrexate may be effective
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
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
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.