A pathogenic role of type I IFN in CANDLE/PRAAS has been validated by blocking type I IFN signaling with Janus kinase (JAK) 1/2 (JAK1/2)-specific tyrosine kinase inhibitors, which results in clinical remission in 50% of patients associated with normalization of the IFN scores in these patients with mostly PSMB8 mutations (21, 22). Here, IFNA1 is linked to proteosome-associated autoinflammatory syndrome.