Macrophage Activation Syndrome

Hematol Oncol Clin North Am. 2015 Oct;29(5):927-41. doi: 10.1016/j.hoc.2015.06.010. Epub 2015 Aug 25.


Macrophage activation syndrome (MAS) is a potentially life-threatening complication of rheumatic disorders that occurs most commonly in systemic juvenile idiopathic arthritis. In recent years, there have been several advances in the understanding of the pathophysiology of MAS. Furthermore, new classification criteria have been developed. Although the place of cytokine blockers in the management of MAS is still unclear, interleukin-1 inhibitors represent a promising adjunctive therapy, particularly in refractory cases.

Keywords: Hemophagocytic lymphohistiocytosis; Hemophagocytic syndrome; Interleukin-1 inhibitors; Macrophage activation syndrome; Proinflammatory cytokines; Systemic juvenile idiopathic arthritis.

Publication types

  • Review

MeSH terms

  • Disease Management
  • Humans
  • Macrophage Activation Syndrome / diagnosis*
  • Macrophage Activation Syndrome / epidemiology
  • Macrophage Activation Syndrome / etiology*
  • Macrophage Activation Syndrome / therapy
  • Practice Guidelines as Topic
  • Risk Factors