More recently, a functional variant in DNASE1L3 was implicated in a familial form of SLE [33], and a pan-meta-analysis of SLE and SSc also confirmed the locus near PXK, but in particular for ACA-positive SSc (rs2176082 [7]; P = 1.4 × 10−4 in our data) strengthening the evidence that this locus plays a role in both diseases. Here, DNASE1L3 is linked to systemic sclerosis.