ATA+ patients carry a significantly higher risk of developing interstitial lungdisease (ILD) within the first 5 years of disease onset (up to 80%), whereaspatients with ARA + have a much lower risk of ILD (33%), but the highest risk forscleroderma renal crisis (SRC) (28% at 20 years) compared to other autoantibody subtypes9 (Table 1). This evidence concerns the gene ATM and interstitial lung disease.