Interleukin (IL)-1 participates in PA remodeling and inflammation during PH by binding to the receptor (IL1R1) which is upregulated and recruiting myeloid differentiation primary response protein 88 (MyD88), which induced IL-1, IL-6, and TNF-α increased through NF-κB activation in patients with IPAH and hypoxia-induced mice (44). Here, MYD88 is linked to idiopathic pulmonary arterial hypertension.