This update includes AR and AD forms of IKZF2 (HELIOS) [20–23] and IL6ST [31–33] deficiency, as well as AD RIPK1 LOF [85, 86], AR GOF TMEM173/STING [70], AR LOF TBK1 [82], and mono-allelic IKZF1 GOF [52] variants which complement previous reports of AR RIPK1 deficiency, AD GOF TMEM173/STING, AD TBK1 deficiency, and mono-allelic IKZF1 inactivating variants, respectively [5]. Here, IL6ST is linked to Alzheimer disease.