MAS/sJIA (MS) score, which distinguishes between MAS and sJIA flare

Clinical information	Points	Beta coefficient (multiplication factor)
CNS involvement (lethargy, seizures, irritability, confusion, headache, mood changes or coma)	1 = present 0 = absent	× 2.44
Hemorrhagic manifestations (petechiae, ecchymoses or purpura, mucosal or gastrointestinal bleeding, or intravascular coagulation)	1 = present 0 = absent	× 1.54
Active arthritis	1 = present 0 = absent	× –1.30
Platelet count	N (× 10⁹/L)	× –0.003
Lactic dehydrogenase	(units/L)	× 0.001
Fibrinogen	N (mg/dL)	× –0.004
Ferritin	N (ng/mL)	× 0.0001
Total score (after points are multiplied by the respective beta coefficient)

Note: The presence of fever is mandatory for this tool.
A total score of ≥2.1 is positive and suggests the presence of MAS in a patient with sJIA.

MAS: macrophage activation syndrome; sJIA: systemic juvenile idiopathic arthritis; CNS: central nervous system.

Adapted with permission from: Minoia F, Bovis F, Davis S, et al. Development and initial validation of the MS score for diagnosis of macrophage activation syndrome in systemic juvenile idiopathic arthritis. Ann Rheum Dis 2019; 78(10):1357-1362. Copyright © 2019 BMJ Publishing Group Ltd.

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MAS/sJIA (MS) score, which distinguishes between MAS and sJIA flare