Table 2 Classification [5] and diagnostic [25] criteria for macrophage activation syndrome (MAS) in systemic juvenile idiopathic arthritis
Parameter | 2016 sJIA/MAS [5] | MAS/sJIA score [25] |
|---|---|---|
Fever | Not specified | – |
Ferritin | >684 ng/mL | 0.0001* serum level |
Platelet count | Platelets ≤ 181,000/mm³ | −0.003* platelet count |
Hemorrhagic manifestations | – | 1.54*1 (yes) or *0 (no) |
Fibrinogen level | ≤360 mg/dL | −0.004* serum level |
LDH | – | 0.001* serum level |
AST | >48 units/L | – |
Triglycerides | >156 | – |
Central nervous system | – | 2.44 *1 (yes) or *0 (no) |
Active arthritis | – | −1.3 *1 (yes) or *0 (no) |
Diagnosis: | Ferritin >684 + 2 criteria (platelet, AST, triglycerides, or fibrinogen) | Sum of parameters ≥ −2.1 |