Pulmonary vasculitis with diffuse alveolar haemorrhage is seen in a third ofSSc-AAV patients, while up to 80% of patients develop ILD (often non-specificinterstitial pneumonitis).23,32 The Australian cohortreported an independent association of ILD with SSc-AAV, after controlling forthe ATA+,22 whereas, anti-PR3+ was not associated with ILD. The gene discussed is PRTN3; the disease is systemic sclerosis.