Factors that elevate the risk of PAH in individuals with SSc include advanced age, prolonged disease duration, reduced DLCO in the absence of substantial PFT impairment (FVC/DLCO > 1.6 in ILD), elevated levels of brain natriuretic peptide (BNP) or N-terminal pro brain natriuretic peptide (NT-proBNP), increased uric acid levels, limited cutaneous SSc subtype (lcSSc), as well as the presence of anti-centromere antibodies and telangiectasias [34,35]. Here, NPPB is linked to pulmonary arterial hypertension.