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-2004^a	MAS 2016^b	MS score^c	Ferritin:ESR ratio^d
Laboratory parameters
Ferritin, μg/L	≥500	>684	Included	Included
Platelets, ×10^9/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, ×10^9/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].