ANA positivity is reported in 78–97% of patients in the jSSc cohorts (6, 9–13), with scleroderma-associated antibodies reflecting the majority of extractable nuclear antigens causing ANA reactivity, such as ATA (Scl-70), centromere, and U1-RNP as reported in adult SSc, but in divergent frequencies, mirroring the slightly different clinical phenotype in children with SSc (Table 2). This evidence concerns the gene ATM and systemic sclerosis.