Macrophage activation syndrome in pediatrics

Pediatr Allergy Immunol. 2020 Feb:31 Suppl 24:13-15. doi: 10.1111/pai.13158.

Abstract

Macrophage activation syndrome (MAS) is a serious, potentially life-threatening, hyperinflammatory condition, which belongs to the spectrum of hemophagocytic lymphohistiocytosis (HLH) and can complicate several immunologic and rheumatic disorders. MAS is characterized by a dysfunctional immune response that is similar to that seen in other forms of HLH. Because MAS may pursue a rapidly fatal course, prompt recognition of its clinical and laboratory features and immediate therapeutic intervention are fundamental. Recently, a set of classification criteria for MAS complicating sJIA has been developed through a multinational collaborative effort. High-dose parenteral corticosteroids remain the mainstay of treatment of MAS.

Keywords: activation; inflammation; macrophage.

MeSH terms

  • Adrenal Cortex Hormones / therapeutic use*
  • Child
  • Cytokines / metabolism
  • Fever
  • Humans
  • Inflammation Mediators / metabolism
  • Lymphocyte Activation
  • Lymphohistiocytosis, Hemophagocytic / diagnosis*
  • Lymphohistiocytosis, Hemophagocytic / drug therapy
  • Lymphohistiocytosis, Hemophagocytic / immunology
  • Macrophage Activation Syndrome / diagnosis*
  • Macrophage Activation Syndrome / drug therapy
  • Macrophage Activation Syndrome / immunology
  • Macrophages / immunology*
  • Multiple Organ Failure
  • T-Lymphocytes / immunology*

Substances

  • Adrenal Cortex Hormones
  • Cytokines
  • Inflammation Mediators