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Macrophage activation syndrome in pediatrics
Author(s) -
Alongi Alessandra,
Naddei Roberta,
De Miglio Laura,
Natoli Valentina,
Ravelli Angelo
Publication year - 2020
Publication title -
pediatric allergy and immunology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.269
H-Index - 89
eISSN - 1399-3038
pISSN - 0905-6157
DOI - 10.1111/pai.13158
Subject(s) - macrophage activation syndrome , medicine , hemophagocytic lymphohistiocytosis , dysfunctional family , immunology , hemophagocytosis , intervention (counseling) , macrophage , immune system , pediatrics , intensive care medicine , arthritis , psychiatry , bone marrow , biochemistry , chemistry , disease , pancytopenia , in vitro
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.