According to 2013 American College of Rheumatology recommendations for screening and monitoring of PAH in rheumatic diseases [144], all SSc patients, patients having SSc spectrum disorders (having sclerodactyly, nailfold capillary abnormalities, or SSc-specific autoantibodies), or those with PAH signs/symptoms (dyspnea on rest/exercise, fatigue, presyncope/syncope, chest pain, palpitations, dizziness, and lightheadedness) should undergo screening evaluations for PAH using PFTs with DLCO, echocardiography, and NT-proBNP. This evidence concerns the gene NPPB and pulmonary arterial hypertension.