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We read with great interest the article by Minoia et al which named development and initial validation of the macrophage activation syndrome (MAS)/systemic juvenile idiopathic arthritis (sJIA) (MS) score for diagnosis of MAS in sJIA.
MAS is a life-threatening complication of rheumatic disorders, including sJIA, adult-onset Still’s disease (AOSD) and lupus.1–4 Timely diagnosis and appropriate treatment of MAS are particularly important to improve the prognosis of MAS patients. At present, hemophagocytic lymphohistiocytosis (HLH)-2004 and HLH-2009 criteria are widely used to identify MAS associated with AOSD. Hemophagocytic syndrome diagnostic (HS) score was developed previously to facilitate MAS recognition, but still requires validation.5 In 2019, Francesca Minoia et al reported a MS score for classification of sJIA-associated MAS patients.6 Considering that sJIA and AOSD are thought to constitute …
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