In the case of APLAID (caused by a p.Ser707Tyr substitution), the auto-inflammation has been suggested to be partially driven by PLCɣ2-dependent activation of the pyrin (PYD)-domain-containing protein 3 (NLRP3) inflammasome [5]. Here, MEFV is linked to autoinflammation-PLCG2-associated antibody deficiency-immune dysregulation.