Evaluation of Macrophage Activation Syndrome in Patients with Systemic Juvenile Idiopathic Arthritis: A Single Center Experience
Table 1
Comparison of HLH-2004 diagnostic guidelines, MAS 2016 classification criteria, MS score, and ferritin to erythrocyte sedimentation rate ratio for the diagnosis of MAS.
HLH-2004a
MAS 2016b
MS scorec
Ferritin:ESR ratiod
Laboratory parameters
Ferritin, μg/L
≥500
>684
Included
Included
Platelets, ×109/L
<100
≤181
Included
—
Aspartate aminotransferase, U/L
—
>48
—
—
Lactate dehydrogenase
—
—
Included
—
Triglycerides, mmol/L
≥3.00
>1.76
—
—
Fibrinogen, μmol/L
≤4.41
≤10.6
Included (mg/dL)
—
Hemoglobin, mmol/L
<5.59
—
—
—
Neutrophils, ×109/L
<1.0
—
—
—
Erythrocyte sedimentation rate, mm
—
—
—
Included
Clinical signs
Fever
Included
Included
Included
Included
CNS involvement
—
—
Included
—
Splenomegaly
Included
—
—
—
Haemorrhagic manifestations
—
—
Included
—
Arthritis
—
—
Included
—
Others
Hemophagocytosis in bone marrow or spleen or lymph nodes
Included
—
—
—
Low or absent NK-cell activity
Included
—
—
—
Soluble CD25 (i.e., soluble IL-2 receptor), U/mL
≥2,400
—
—
—
A molecular diagnosis consistent with HLH
Included
—
—
—
aHLH-2004 criteria includes molecular diagnosis consistent with HLH or 5 of 8 criteria: fever, splenomegaly, cytopenia of 3 lineages ( mmol/L, /L, /L), mmol/L, μmol/L, hemophagocytosis, low NK-cell activity, μg/L, and soluble U/mL [15]. bMAS 2016 criteria include fever and μg/L plus 2 of 4 criteria: /L, U/L, mmol/L, and μmol/L [7]. cCalculation of the MS score: [16]. dFerritin/ESR considered as a screening tool for the diagnosis of MAS [17].