Abstract
Macrophage activation syndrome (MAS) is a life-threatening systemic immune-mediated condition and a catastrophic complication of autoinflammatory conditions such as adult-onset Still’s disease (AOSD) and systemic juvenile idiopathic arthritis (SJIA). It is a subtype of hemophagocytic lymphohistiocytosis (HLH) associated with underlying rheumatologic conditions, both autoimmune and autoinflammatory. The patients are often acutely ill from an ongoing “cytokine storm,” and there is significant morbidity and mortality associated with it. Aggressive treatment with high-dose intravenous corticosteroids, immunomodulators, antineoplastic agents, and, depending on the underlying etiology, biologics is necessary to prevent organ damage and improve survival.
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Mehta, B., Efthimiou, P. (2019). Macrophage Activation Syndrome (MAS). In: Efthimiou, P. (eds) Auto-Inflammatory Syndromes. Springer, Cham. https://doi.org/10.1007/978-3-319-96929-9_14
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DOI: https://doi.org/10.1007/978-3-319-96929-9_14
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