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. Author manuscript; available in PMC: 2015 Apr 1.
Published in final edited form as: Nat Genet. 2014 Sep 14;46(10):1140–1146. doi: 10.1038/ng.3089

Figure 1. Severe flares in a patient with an NLRC4 mutation are consistent with Macrophage Activation Syndrome.

Figure 1

(a) and (b) Evanescent, urticaria-like rashes with dermographism during flares. (c) Sonographic measurement of splenomegaly with longest dimension equivalent to an age-matched z-score of 3.3239. (d) H&E micrograph of duodenal mucosal biopsy obtained at 18 months of age for vomiting and poor weight gain (20× magnification) showing non-specific mixed inflammatory infiltrate in the lamina propria and mild villous blunting. (e) Laboratory markers used to monitor Macrophage Activation Syndrome (MAS). The C-reactive Protein (CRP) is elevated, Hemoglobin (Hgb) is depressed, Platelet count is depressed, and serum ferritin rises during flares of MAS. All of the patient’s available laboratory data since birth are plotted (see also Supplementary Table 4). Pink bars indicate severe disease flares. Dashed lines indicate the normal ranges. The x-axis indicates the patient’s age in years (non-linear).