SGCB and pulmonary arterial hypertension: According to the 2017 EULAR recommendations [55], ERA (ambrisentan, bosentan, and macitentan), PDE5i (sildenafil and tadalafil), and an sGC stimulator (riociguats) are considered the first-line options for treating SSc-PAH based on high-quality RCTs that showed improvement in exercise capacity, the time to clinical worsening (defined as a composite of death, hospitalization, and disease progression), and/or PAH-related hemodynamics in heterogeneous patients with PAH including RD-PAH.