The latter together with immunological findings and the infection-exacerbated disease course, suggest dysregulated innate immune sensing and the consequent overproduction of proinflammatory cytokines as the mechanism of systemic autoinflammation in patients with hemizygote loss-of-function mutations in ELF4. 29Ineffective handling of infectious agents, especially viruses,31 may be also relevant for enhanced innate immune activation in ELF4 deficiency. The gene discussed is ELF4; the disease is infection.