Macrophage activation syndrome in pediatrics.

Alessandra Alongi, Roberta Naddei, Laura De Miglio, Valentina Natoli, Angelo Ravelli

Pediatric allergy and immunology : official publication of the European Society of Pediatric Allergy and Immunology 2020 Feb

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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. © 2020 EAACI and John Wiley and Sons A/S. Published by John Wiley and Sons Ltd.

Citation

Alessandra Alongi, Roberta Naddei, Laura De Miglio, Valentina Natoli, Angelo Ravelli. Macrophage activation syndrome in pediatrics. Pediatric allergy and immunology : official publication of the European Society of Pediatric Allergy and Immunology. 2020 Feb;31 Suppl 24:13-15