The serological profile in SSc patients plays a significant role in the prognosis of the current ILD: anti-topoisomerase I (ATA; anti-Scl-70) antibodies [11,12] are predominantly associated with the development of pulmonary fibrosis, while anti-U3 RNP antibodies are linked to a higher incidence of pulmonary hypertension and cardiac involvement [3]. This evidence concerns the gene ATM and pulmonary arterial hypertension.