Table 1.

Clinical and preclinical evidence for inflammation in pulmonary hypertension

Target	Preclinical	Clinical
NF‐κB	Inhibition improves PH (Kumar et al., 2012; Hosokawa et al., 2013; Wang et al., 2013; Farkas et al., 2014; Li et al., 2014)	–
IL‐1	Inhibition improves PH (Voelkel et al., 1994; Parpaleix et al., 2016)	Increased levels in patients (Humbert et al., 1995; Soon et al., 2010)
IL‐18	Inhibition improves PH (Morisawa et al., 2016)	Increased levels in patients (Ross et al., 2012)
IL‐6	Inhibition improves PH (Golembeski et al., 2005; Savale et al., 2009; Steiner et al., 2009; Hashimoto‐Kataoka et al., 2015; Parpaleix et al., 2016)	Increased levels in patients (Humbert et al., 1995; Selimovic et al., 2009; Soon et al., 2010; Gitto et al., 2012; Matura et al., 2015)
Other cytokines/chemokines/growth factors	–	Increased levels of MCP‐1 (Itoh et al., 2006); VEGF, PDGF, TGF‐β1 (Selimovic et al., 2009); IL‐2, ‐4, ‐8, ‐10, ‐12p70, TNF‐α (Soon et al., 2010) IL‐8, TNF‐α in PPHN (Gitto et al., 2012); CXCL10 (Ross et al., 2012); TNF‐α (Matura et al., 2015)
Inflammatory cells	Depletion of T‐cells worsens PH (Tamosiuniene et al., 2011; Chu et al., 2015); macrophages improves PH (Zaloudikova et al., 2016)	Infiltrates comprised of macrophages, T and B lymphocytes, dendritic and mast cells (Tuder et al., 1994; Savai et al., 2012; Stacher et al., 2012)
P2X7/ATP	Inhibition improves PH (Yin et al., 2017)	–
Inflammasomes	Inhibition improves PH (Villegas et al., 2013; Cero et al., 2015; Tang et al., 2015)	–
Survival	–	Elevated cytokines predict (Soon et al., 2010)
Remodelling	–	Inflammatory infiltrates correlate with vessel wall thickness (Stacher et al., 2012)