Macrophage activation syndrome in pediatrics
- PMID: 32017214
- DOI: 10.1111/pai.13158
Macrophage activation syndrome in pediatrics
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.
© 2020 EAACI and John Wiley and Sons A/S. Published by John Wiley and Sons Ltd.
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