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Table 2 Preliminary diagnostic guidelines for MAS complicating sJIA [3]

From: Evidence-based diagnosis and treatment of macrophage activation syndrome in systemic juvenile idiopathic arthritis

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)

 
  1. 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
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Pediatric Rheumatology

ISSN: 1546-0096