Using genetic and pharmacological approaches, we identify G-CSF as the key driver of autoinflammation in APLAID caused by missense mutations in PLCG2. Allogeneic BM transplantation may be beneficial in individuals with APLAID as indicated by our preclinical dataset. The gene discussed is PLCG2; the disease is autoinflammation-PLCG2-associated antibody deficiency-immune dysregulation.