Predictors of progression to SSc-PAH from SSc-AR-PAH (BNP: HR (95% CI) 0.6 (0.1–5.7); NT-proBNP: 1.6 (0.2–14.3), composite BNP/NT-proBNP group predicted mortality (HR 3.81 (2.08–6.99), p < 0.0001). The gene discussed is NPPB; the disease is pulmonary arterial hypertension.