Supplementary Figure 1: Supporting clinical information.
(a) The patient’s growth chart. (b) Additional parameters associated with macrophage activation syndrome (MAS) reflecting all of the patient’s available laboratory data (see also Supplementary Table 1). Pink bars indicate severe disease flares, and the blue bar indicates testing done after IL-1 receptor antagonist treatment (anakinra). The dashed lines indicate the normal ranges. The dotted lines indicate the patient’s age in years. ESR, erythrocyte sedimentation rate; WBC, white blood cell count; ALT, alanine aminotransferase; LDH, lactate dehydrogenase. (c) Sonographic measurement of the patient’s spleen during a flare before the initiation of treatment with anakinra (left; z score = 3.95) and 3 months after the initiation of anakinra (right; z score = 1.3)32.
