Ifetroban, a thromboxane A2/prostaglandin H2 receptor antagonist, and bardoxolone, an inductor of the nuclear factor erythroid 2-related factor 2 (Nrf2) and suppressor of the nuclear factor-kB (NF-kB), are currently under investigation for the treatment of CTD-PAH [49]. Here, NFKB1 is linked to pulmonary arterial hypertension.