As per the American College of Rheumatology (ACR) 2013 recommendations regarding clinical monitoring and screening for PAH in rheumatological disorders, screening evaluation should be done with echocardiography, NT-pro BNP, DLCO, and PFT in all systemic sclerosis patients who have clinical features such as abnormalities in nail fold capillaries, sclerodactyly, presyncope, dyspnea on exertion, and fatigue, as well as those patients with positive auto-antibodies specific to SSc [98]. Here, NPPB is linked to systemic sclerosis.