Table 2.

Preliminary diagnostic guidelines for MAS complicating sJIA [3]

Clinical criteria
1. Central nervous system dysfunction
2. Haemorrhages
3. Hepatomegaly
Laboratory criteria
1. Decreased platelets count (≤262 × 109/L)
2. Elevated levels of aspartate aminotransferase (>59 U/L)
3. Decreased white blood cell count (≤4.0 × 109/L)
4. Hypofibrinogenemia (≤2.5 g/L)

Presence of any 2 or more laboratory or of any 2 or more clinical and/or laboratory criteria is required for the diagnosis of MAS in sJIA