As in patients with PAH in general, patients with SLE-PAH who respondwell to pulmonary vasodilator therapy (i.e., achieving New York Heart Association (NYHA) functional classI-II, normalization of BNP and RV function, and increase in 6MWD) have a betterprognosis than those who do not [81]. Here, NPPB is linked to pulmonary arterial hypertension.