In addition, increased production of IL-6 by patient-derived PBMCs and CD14-positive macrophages differentiated from PBMCs stimulated with IL-1β was observed (Fig. 2, C and D), confirming previous reports of some ORAS patients (Zhou et al., 2016). Here, CD14 is linked to autoinflammation, panniculitis, and dermatosis syndrome, autosomal recessive.