IL-1α expression in PBMCs in 3 ECD patients and 5 healthy controls (CT). (A) Results of flow cytometric analysis for 1 healthy control and the 3 ECD patients. For patient 1, analysis was performed before and 3 months after the end of anakinra treatment, and for patient 2, analysis was conducted before and after 11 months of ongoing treatment. Patient 3 had no treatment. The first column shows the percentage of CD14⁺CD4^dim PBMCs. The second column represents membranous IL-1α expression (colored histogram) compared with the control isotype, with the mean fluorescence intensity (MFI) of each antibody. Column 4 shows the percentage of IL-1α–positive monocytes, and column 3 shows that of the control isotype in the same cells. (B-C) Respectively, the percentage of IL-1α–positive monocytes and the MFI of IL-1α on monocytes for the 5 healthy controls compared with that of the 2 ECD patients before and after treatment with anakinra (patients 1 and 2) and the untreated patient 3. The final levels on (B) and (C) were calculated by subtracting the measured levels of isotype from those of IL-1α on (A); second column for the MFI; third and fourth columns for the percentage.