Treatment of Systemic Onset Juvenile Rheumatoid Arthritis
Treatment of Systemic Onset Juvenile Rheumatoid Arthritis
What is the role of methotrexate (MTX) in controlling manifestations of systemic onset juvenile rheumatoid arthritis (JRA)? The patient is a 6-year-old boy with fever and rashes who has failed to respond to indomethacin and corticosteroids, although his arthritis had subsided. I'm debating whether intravenous immunoglobulin (IVIG) or MTX should be the next step in treatment.
Arnon Ya'ari, MD
Still's disease, or systemic JRA, makes up roughly 20% of the JRA population. It has the key manifestations of quotidian fevers, evanescent rashes, and arthritis. The diagnosis of systemic JRA is supported by findings of serositis, RES involvement (hepatosplenomegaly [HSM], increased LFTs, or lymphadenopathy), prodromal sore throat (more common in adults), myalgias, wrist involvement, polyarthritis, weight loss, leukocytosis with left shift, anemia of chronic disease, very high acute-phase reactants (erythrocyte sedimentation rate [ESR], C-reactive protein, ferritin), hypoalbuminemia, and seronegative results for antinuclear antibodies and rheumatoid factor. Treatment mainstays include high-dose indomethacin, intermediate- to high-dose corticosteroids (low dose is seldom effective) and, if necessary, disease-modifying antirheumatic drugs (methotrexate, azathioprine, or hydroxychloroquine) or tumor necrosis factor-alpha (TNF-alpha) inhibitors.
For patients such as yours, with persistent systemic disease (fever, rash, HSM, serositis, high ESR), the most effective therapies include prednisone (usually 1-2 mg/kg), methotrexate weekly (in doses similar to that used in JRA), or a TNF-alpha inhibitor. The use of the latter is perplexing, as most research has shown many of the manifestations of Still's disease to be closely linked to cytokines, especially interleukin 6. Thus, the positive effects of either etanercept or infliximab are expected for the arthritis of Still's disease, but not necessarily for the disease's systemic manifestations. Nonetheless, these TNF-alpha inhibitors also have been efficacious in treating patients such as yours. I would not advocate the use of IVIG, because the benefits of this expensive modality have not been well documented in systemic JRA.
What is the role of methotrexate (MTX) in controlling manifestations of systemic onset juvenile rheumatoid arthritis (JRA)? The patient is a 6-year-old boy with fever and rashes who has failed to respond to indomethacin and corticosteroids, although his arthritis had subsided. I'm debating whether intravenous immunoglobulin (IVIG) or MTX should be the next step in treatment.
Arnon Ya'ari, MD
Still's disease, or systemic JRA, makes up roughly 20% of the JRA population. It has the key manifestations of quotidian fevers, evanescent rashes, and arthritis. The diagnosis of systemic JRA is supported by findings of serositis, RES involvement (hepatosplenomegaly [HSM], increased LFTs, or lymphadenopathy), prodromal sore throat (more common in adults), myalgias, wrist involvement, polyarthritis, weight loss, leukocytosis with left shift, anemia of chronic disease, very high acute-phase reactants (erythrocyte sedimentation rate [ESR], C-reactive protein, ferritin), hypoalbuminemia, and seronegative results for antinuclear antibodies and rheumatoid factor. Treatment mainstays include high-dose indomethacin, intermediate- to high-dose corticosteroids (low dose is seldom effective) and, if necessary, disease-modifying antirheumatic drugs (methotrexate, azathioprine, or hydroxychloroquine) or tumor necrosis factor-alpha (TNF-alpha) inhibitors.
For patients such as yours, with persistent systemic disease (fever, rash, HSM, serositis, high ESR), the most effective therapies include prednisone (usually 1-2 mg/kg), methotrexate weekly (in doses similar to that used in JRA), or a TNF-alpha inhibitor. The use of the latter is perplexing, as most research has shown many of the manifestations of Still's disease to be closely linked to cytokines, especially interleukin 6. Thus, the positive effects of either etanercept or infliximab are expected for the arthritis of Still's disease, but not necessarily for the disease's systemic manifestations. Nonetheless, these TNF-alpha inhibitors also have been efficacious in treating patients such as yours. I would not advocate the use of IVIG, because the benefits of this expensive modality have not been well documented in systemic JRA.
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