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MODERN RHEUMATOLOGY Vol.14 No.1
>MR14-1
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Inflammatory cytokines and systemic-onset juvenile idiopathic arthritis
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| Shumpei Yokota1 , Takako Miyamae1, Tomoyuki Imagawa1, Naomi Iwata1, 2, Shigeki Katakura1 and Masaaki Mori1 |
(1) Department of Pediatrics, Yokohama City University School of Medicines, 3-9 Fukuura, Kanazawa-ku, Yokohama, 236-0004, Japan
(2) Department of Rheumatic Diseases, Aichi Childrens Health and Medical Center, Obu, Japan |
Received: 22 April 2003 Accepted: 07 August 2003 |
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Abstract |
| Abstract Systemic-onset juvenile idiopathic arthritis (JIA) is a severe and steroid-dependent disease, which sometimes progresses to the fatal disease macrophage activation syndrome. An investigation of inflammatory cytokine levels revealed increases in IL-6 in serum of systemic-onset disease patients. Continuously elevated levels of IL-6 in serum may play a important role in manifesting the clinical symptoms and signs of systemic-onset JIA, including spiking fever, rash, arthritis, and serositis. The characteristic fever spikes parallel IL-6 levels. Long-term exposure to high levels of IL-6 in children results in severe growth impairment, which was strongly suggested by the recent establishment of IL-6 transgenic mice. To avoid disease progression to macrophage activation syndrome and the adverse effects of high-dose corticosteroids, it might be reasonable to inhibit the formation of IL-6/IL-6R complex in order to block the binding to gp130 receptor, a biologically active receptor for IL-6. This review will provide evidence of the relationship between IL-6 homeostasis and systemic-onset JIA, and our recent trials of anti-IL-6R antibody (MRA) for children with acute systemic disease intractable to long-term and high-dose corticosteroid therapy. MRA could be a therapeutic modality for children with systemic-onset JIA intractable to high-dose corticosteroids. |
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| Key words |
| Key words Antirheumatic drug - Cytokine - Cytokine-inducible proteins - Inflammation - Juvenile idiopathic arthritis (JIA) |
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