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Be alert for macrophage activation syndrome in seriously sick children with underlying pro-inflammatory disorders

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Abstract

Macrophage activation syndrome (MAS) is a serious complication of pro-inflammatory conditions, such as systemic juvenile idiopathic arthritis. MAS results from over-stimulated immune-related cytokines. Now considered a form of acquired haemophagocytic lymphohistiocytosis, MAS is fatal in up to 20% of children. Fast diagnosis and immediate treatment with broad immunosuppressants and biological drugs targeting inflammatory cytokines are required. High ferritin levels and a high ferritin to erythrocyte sedimentation rate ratio are quick indicators of potential MAS.

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    Caroline Fenton & Young-A Heo

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  1. Caroline Fenton
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Correspondence to Young-A Heo.

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CF searched the literature, prepared the bibliography and wrote the first draft. YAH contributed to the content and revision of the article.

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CF and YAH declare no relevant conflicts of interest.

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The preparation of this review was not supported by any external funding. CF and YAH are employed by Adis International Ltd./Springer Nature.

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Fenton, C., Heo, YA. Be alert for macrophage activation syndrome in seriously sick children with underlying pro-inflammatory disorders. Drugs Ther Perspect 36, 280–285 (2020). https://doi.org/10.1007/s40267-020-00746-6

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