Criteria
Macrophage activation syndrome
A life-threatening hyperinflammatory complication of systemic juvenile idiopathic arthritis, caused by uncontrolled macrophage and T-cell activation with falling blood counts and very high ferritin.
hyperinflammatory complication of systemic JIA
Also called: MAS
Criteria not met
1 gate + 1 ferritin + 2 lab
Educational use only. Guideline: 2016 EULAR/ACR/PRINTO classification criteria, febrile systemic JIA patient. Source: Ravelli A, Minoia F, Davì S, et al. 2016 Classification Criteria for Macrophage Activation Syndrome Complicating Systemic Juvenile Idiopathic Arthritis. Ann Rheum Dis. 2016. https://pubmed.ncbi.nlm.nih.gov/26865703/; Macrophage Activation Syndrome. Merck Manuals Professional Edition. 2025. https://www.merckmanuals.com/professional/pediatrics/rheumatologic-disorders-in-children/macrophage-activation-syndrome; Schulert GS, Grom AA. Pathogenesis of macrophage activation syndrome and potential for cytokine-directed therapies. Annual Review of Medicine. 2015. https://pmc.ncbi.nlm.nih.gov/articles/PMC5846123/.