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Macrophage Activation Syndrome (MAS)

  • Bella Mehta
  • Petros Efthimiou
Chapter

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.

Keywords

Macrophage activation syndrome Reactive hemophagocytosis syndrome Hyperferritinemia Cytopenia Cytokine storm Persistent macrophage activation 

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Copyright information

© Springer Nature Switzerland AG 2019

Authors and Affiliations

  • Bella Mehta
    • 1
    • 2
  • Petros Efthimiou
    • 3
    • 4
  1. 1.Weill Cornell MedicineNew YorkUSA
  2. 2.Hospital for Special SurgeryNew YorkUSA
  3. 3.New York University School of MedicineNew YorkUSA
  4. 4.NYU/Langone HealthNew YorkUSA

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