Criteria for Cytokine Storm Syndromes

Abstract

In the past two decades, there has been a great deal of work aimed to devise diagnostic guidelines, classification criteria, and diagnostic scores for cytokine storm syndromes. The most notable effort has been the large-scale multinational study that led to the development of the 2016 classification criteria for macrophage activation syndrome (MAS) complicating systemic juvenile idiopathic arthritis (JIA). In the context of this project, the laboratory tests whose change over time is most valuable for early diagnosis of MAS in systemic JIA were identified. Other tools are the MH score to distinguish MAS from primary hemophagocytic lymphohistiocytosis, the diagnostic guidelines for MAS in childhood-onset systemic lupus erythematosus, the HScore for reactive hemophagocytic syndromes, and the MS score for early detection of MAS in systemic JIA. Future studies should scrutinize the validity of proposed criteria in different patient populations evaluated prospectively. There is also the specific need to assess the diagnostic performance of criteria in patients with systemic JIA treated with biologics.