2014•Pediatric RheumatologyOpen access

Macrophage activation syndrome in patients with systemic juvenile idiopathic arthritis treated with tocilizumab

Fabrizio De Benedetti, Rayfel Schneider, Sheila S Weitzman, Clare E. Devlin, Kaori Daimaru, Shumpei Yokota, Syuji Takei, Angelo Ravelli

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Abstract

Macrophage activation syndrome (MAS) is a severe, potentially fatal complication of systemic juvenile idiopathic arthritis (sJIA). Changes in therapies, including biologics, have been associated with the onset of MAS. Interleukin-6 (IL-6) plays a major pathogenic role in sJIA; data in animals suggest that high IL-6 levels contribute to the triggering of MAS [ 1 ]. Treatment with the IL-6 receptor inhibitor tocilizumab (TCZ) is highly effective in patients with sJIA [ 2 ].

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What this paper is about

Macrophage activation syndrome (MAS) is a severe, potentially fatal complication of systemic juvenile idiopathic arthritis (sJIA). Changes in therapies, including biologics, have been associated with the onset of MAS. Interleukin-6 (IL-6) plays a major pathogenic role in sJIA; data in animals suggest that high IL-6 levels contribute to the triggering of MAS [ 1 ]. Treatment with the IL-6 receptor inhibitor tocilizumab (TCZ) is highly effective in patients with sJIA [ 2 ].

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Available abstract

Macrophage activation syndrome (MAS) is a severe, potentially fatal complication of systemic juvenile idiopathic arthritis (sJIA). Changes in therapies, including biologics, have been associated with the onset of MAS. Interleukin-6 (IL-6) plays a major pathogenic role in sJIA; data in animals suggest that high IL-6 levels contribute to the triggering of MAS [ 1 ]. Treatment with the IL-6 receptor inhibitor tocilizumab (TCZ) is highly effective in patients with sJIA [ 2 ].

Key concepts: Tocilizumab, Macrophage activation syndrome, Medicine, Rheumatology, Arthritis, Juvenile, Interleukin 6, Internal medicine

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