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Macrophage Activation Syndrome (MAS)

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Auto-Inflammatory Syndromes

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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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Authors and Affiliations

  1. Weill Cornell Medicine, New York, NY, USA

    Bella Mehta

  2. Hospital for Special Surgery, New York, NY, USA

    Bella Mehta

  3. New York University School of Medicine, New York, NY, USA

    Petros Efthimiou

  4. NYU/Langone Health, New York, NY, USA

    Petros Efthimiou

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  1. Bella Mehta
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  1. New York University School of Medicine, New York, NY, USA

    Petros Efthimiou

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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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