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Systemic Juvenile Idiopathic Arthritis – New Aspects of Clinical Features, Diagnostic Tools and Treatment Strategies

Elke Lainka, Johannes Peter Haas, Gerd Horneff, Elisabeth Weißbarth-Riedel, Tim Niehues, Helmut Wittkowski, Ulrich Neudorf.

Systemic juvenile idiopathic arthritis (SJIA) is an autoinflammatory disease (AID) of unknown etiology related to abnormalities in the innate immune system. The pathogenesis of SJIA differs from all other forms of JIA, which are supposed to be autoimmune diseases. It does not show any association with HLA genes or autoantibodies. Specific autoimmune reactions of the adaptive immune system as in oligo- or polyarticular juvenile idiopathic arthritis (JIA) are not observed in most SJIA cases. In contrast, the major role in the pathogenesis has been ascribed to proinflammatory cytokines interleukin-1 and interleukin-6. Specific inhibition of these two cytokines represents a novel therapeutic approach to the disease which is expected to improve long term prognosis.
Aims: This overview describes our current understanding of clinical features and pathophysiology, as well as the state of the art in diagnostic procedures and treatment options for SJIA.

Key words: Juvenile idiopathic arthritis, systemic juvenile idiopathic arthritis, autoinflammatory disease, biomarkers, biological agents

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