Gain-of-function mutations of human STING1 cause SAVI, which is characterized by severe interstitial lung disease, T cell lymphopenia, skin inflammation, and perturbed IFN- and NF-κB-driven signaling (Clarke et al., 2020; Liu et al., 2014; Picard et al., 2016; Tang et al., 2020). The gene discussed is STING1; the disease is interstitial lung disease.