Macrophage activation syndrome in pediatrics. (3rd February 2020)

• Record Type: Journal Article
• Authors: Alongi, Alessandra; Naddei, Roberta; De Miglio, Laura; Natoli, Valentina; Ravelli, Angelo
• Is Part Of: Pediatric allergy and immunology. Volume 31(2020)Supplement 24
• Page Start: 13
• Page End: 15
• Publication Date: 2020-02-03
• Subjects: activation -- inflammation -- macrophage; Allergy in children -- Periodicals; Immunologic diseases in children -- Periodicals; 617
• DOI: 10.1111/pai.13158
• Languages: English
• ISSNs: 0905-6157

Abstract

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.