Furthermore, overexpression of IL-6 in mice causes elevated pulmonary vascular remodeling and PAH (Steiner et al., 2009), while IL-6 deficient mice are not susceptible for PH (Savale et al., 2009) and ectopic upregulation of membrane-bound IL-6 receptor (IL-6R) on PA-SMCs rodent models of PH fosters vascular remodeling that can be attenuated with IL6R/sIL6R antagonist ERBF and an IL-6R-neutralizing antibody (Tamura et al., 2018; Figure 1). Here, IL6R is linked to pulmonary arterial hypertension.