NPPB and pulmonary arterial hypertension: Age > 60 years, male sex, systolic blood pressure ≤ 110 mmHg, pericardial effusion, PVR > 32 Woods, DLCO > 39%, poor functional status, 6MWD < 165 m and BNP > 180 pg/mL have been identified as significant predictors of mortality in SSc-PAH patients [14,117,118,119].