In 2012, the Australian Scleroderma Interest Group (ASIG) developed a screening algorithm for SSc-PAH by using serum NT-proBNP level and PFT [11]; this was found to have similar sensitivity and higher specificity and positive (PPV) and negative (NPV) predictive value in comparison with the ESC/ERS guidelines [13]. This evidence concerns the gene NPPB and systemic sclerosis.