Table 2

Univariate analysis of the ability of specific variables to distinguish patients with macrophage activation syndrome from comparison patients, as assessed using the 391 patients for whom expert consensus was achieved

No. of patients with available dataOR (95% CI)p Value
Clinical features
 Central nervous system involvement3858.1 (4.5 to 14.4)<0.0001
 Hepatomegaly3897.7 (4.5 to 12.9)<0.0001
 Haemorrhagic manifestations3866.5 (3.3 to 12.8)<0.0001
 Fever3885.4 (0.7 to 40.9)0.106
 Splenomegaly3864.6 (2.8 to 7.6)<0.0001
 Lymphadenopathy3833.4 (2.1 to 5.5)<0.0001
 Kidney involvement3903.5 (1.7 to 7.4) 0.0008
 Heart involvement3883.1 (1.7 to 5.5) 0.0001
 Lung involvement3892.5 (1.4 to 4.4) 0.0011
 Active arthritis3901.6 (1.0 to 2.6) 0.0587
Laboratory features
Non-normalised values
 Ferritin >684 ng/mL334111.6 (26.7 to 465.8)<0.0001
 Platelet count ≤181×109/L38584.3 (40 to 177.5)<0.0001
 Aspartate aminotransferase >48 units/L37751.9 (21.7 to 124.4)<0.0001
 Lactate dehydrogenase >853 units/L32920.0 (10.7 to 37.3)<0.0001
 Triglycerides >156 mg/dL27219.6 (9.4 to 40.8)<0.0001
 Alanine aminotransferase >36 units/L37518.0 (9.4 to 34.3)<0.0001
 Fibrinogen ≤360 mg/dL31411.5 (6.3 to 21.0)<0.0001
 Neutrophil count ≤3.7×109/L3185.9 (3.4 to 10.5)<0.0001
 D-dimer >1350 ng/mL1425.7 (2.4 to 13.4)<0.0001
 Erythrocyte sedimentation rate ≤30 mm/h3355.6 (3.3 to 9.5)<0.0001
 Albumin ≤3.6 gm/dL3315.5 (3.0 to 10.1)<0.0001
 Haemoglobin ≤8.5 gm/dL3844.9 (2.7 to 8.8)<0.0001
 Leucocyte count ≤10.2×109/L3844.6 (2.8 to 7.5)<0.0001
 Serum sodium ≤133, mEq/L3392.9 (1.7 to 5.0) 0.0002
 C reactive protein >0.86 mg/dl3672.4 (1.0 to 5.9) 0.0501
Normalised values
 Ferritin >2773.6 ng/mL33423.9 (12.7 to 44.8)<0.0001
 Aspartate aminotransferase >44.4 units/L37747.4 (21.6 to 103.9)<0.0001
 Alanine aminotransferase >23 units/L37539.4 (14.1 to 110.5)<0.0001
 Lactate dehydrogenase >238.8 units/L32939.2 (18.2 to 84.6)<0.0001
 Triglycerides >193.9 mg/dL27216.3 (8.7 to 30.8)<0.0001
 Fibrinogen ≤318.9 mg/dL31411.0 (6.1 to 20.0)<0.0001
 Albumin ≤3.9 gm/dL3316.4 (3.6 to 11.3)<0.0001
 D-dimer >1320.3 ng/mL1425.2 (2.1 to 12.7)<0.0001
 Serum sodium ≤135 mEq/L3393.3 (1.9 to 5.6)<0.0001