Finally, LoF variants of the gene coding for the transcription factor CCAAT enhancer binding protein epsilon (C/EBPε) cause a PID called AR neutrophil-specific granule deficiency-1 (SGD) (114), whereas heterozygous GoF variants have been recently related to an autoinflammatory disease called the C/EBPε-associated autoinflammation and immune impairment of neutrophils (CAIN; no. 25 in Table 1 and Figure 1). Here, CEBPE is linked to specific granule deficiency.