Classical IL-6 signaling: a promising therapeutic target for pulmonary arterial hypertension

J Clin Invest. 2018 May 1;128(5):1720-1723. doi: 10.1172/JCI120415. Epub 2018 Apr 9.


Current therapies for pulmonary arterial hypertension (PAH) provide symptomatic relief and improve prognosis but fall short of improving long-term survival. There is emerging evidence for a role of inflammatory mediators, primarily IL-6, in the pathogenesis of PAH. However, the mechanisms by which IL-6 potentially affects PAH are unknown. In this issue of the JCI, Tamura, Phan, and colleagues identified ectopic upregulation of the membrane-bound IL-6 receptor (IL6R), indicating classical IL-6 signaling in the smooth muscle layer of remodeled vessels in human and experimental PAH. They performed a series of in vitro and in vivo experiments that provide deeper insights into the mechanisms of classical IL-6 signaling and propose interventions directed against IL6R as a potential therapeutic strategy for PAH.

Publication types

  • Research Support, Non-U.S. Gov't
  • Comment

MeSH terms

  • Humans
  • Hypertension, Pulmonary*
  • Interleukin-6
  • Receptors, Interleukin-6
  • Up-Regulation
  • Vascular Remodeling*


  • IL6R protein, human
  • Interleukin-6
  • Receptors, Interleukin-6