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). Here, SNRNP70 is linked to systemic sclerosis.