CID-like presentation with PCP and severe T-cell lymphopenia was previously reported in two patients with stimulator of interferon genes (STING)-associated vasculopathy with onset in infancy (SAVI), which is also a type I interferonopathy and is caused by a GOF mutation in the transmembrane protein 173 gene (TMEM173) encoding STING (24, 25). The gene discussed is STING1; the disease is STING-associated vasculopathy with onset in infancy.