The two UNC93B1 variants were nominated as potential effectors for systemic inflammation in our subjects according to the well-known biological role of UNC93B1 in autoimmunity15, predicted pathogenicity scores (Supplementary Table 2) and lack of other likely pathogenic variants found in primary immunodeficiency genes based on another early onset lupus cohort16. This evidence concerns the gene UNC93B1 and inborn error of immunity.