Notch ligands stimulate immune regulatory mechanism of inflammatory cells in PAH

Abstract

Besides vasoconstriction and in situ thrombosis, “vascular remodelling” is a key feature of pulmonary arterial hypertension (PAH). This process is comprised of cellular hypertrophy, cell proliferation in the intima and media of vessels involved. An increased accumulation of T- and B-lymphocytes as well as of macrophages was observed in plexiform lesions and in the remodelled artery walls. Furthermore, elevated levels of pro- and anti-inflammatory cytokines, e.g. IL-6, IL-10, and TNF-α can be found. Additionally, an increase of Notch1 and JAG-1 in pulmonary arteries has been observed.