The extremely rare, dominantly inherited PLCγ2-associated antibody deficiency and immune dysregulation (PLAID, OMIM# 614468) (2, 3) and autoinflammation and PLCγ2-associated antibody deficiency and immune dysregulation(APLAID, OMIM# 614878) (4, 5) are rare monogenic AIDs combining humoral immune deficiency and aseptic inflammation caused by PLCG2 gene mutations. Here, PLCG2 is linked to agammaglobulinemia.