Vascular-Parenchymal Crosstalk Promotes Lung Fibrosis Through BMPR2 Signaling.

Toyoshi YanagiharaKazuya TsubouchiQuan ZhouMichael ChongKohei OtsuboTakuma IsshikiJonas Christian SchuppSeidai SatoCiaran ScallanChandak UpaguptaSpencer RevillAnmar AyoubSy Giin ChongAnna Dvorkin-GhevaNaftali KaminskiJussi TikkanenShaf KeshavjeeGuillaume ParéChristophe GuignabertKjetil Ask Martin R J Kolb

Published in: American journal of respiratory and critical care medicine (2023)

Endothelial dysfunction and vascular remodeling in PH secondary to pulmonary fibrosis enhance fibrogenesis through impaired BMPR2 signaling. Tacrolimus may have value as treatment of advanced IPF and concomitant PH. Genetic abnormalities may determine the development of PH in advanced IPF.

Keyphrases

pulmonary fibrosis
idiopathic pulmonary fibrosis
pulmonary arterial hypertension
genome wide
gene expression
copy number
pulmonary hypertension
dna methylation
replacement therapy