DNASE1L3 and systemic lupus erythematosus: We outline a consistent pattern of early-onset SLE in our affected patients along with biopsy evidence of HUV, in line with several reports in the literature.10, 1223 Most of what has been reported in the literature regarding DNASE1L3 variants were loss-of-function variants.9, 1214 17 Here we report the effect of a novel missense variant (c.572A>G, p.Asn191Ser) in DNASE1L3 on phenotypic SLE with HUV.