Idiopathic pulmonary fibrosis and pulmonary hypertension: Heracles meets the Hydra
Year: 2020
Type: review
Abstract: Idiopathic pulmonary fibrosis (IPF) is a fatal lung disease where the additional presence of pulmonary hypertension (PH) reduces survival. In particular, the presence of coexistent pulmonary vascular disease in patients with advanced lung parenchymal disease results in worse outcomes than either diagnosis alone. This is true with respect to the natural histories of these diseases, outcomes with medical therapies, and even outcomes following lung transplantation. Consequently, there is a striking need for improved treatments for PH in the setting of IPF. In this review, we summarize existing therapies from the perspective of molecular mechanisms underlying lung fibrosis and vasoconstriction/vascular remodelling and discuss potential future targets for pharmacotherapy. LINKED ARTICLES: This article is part of a themed issue on Risk factors, comorbidities, and comedications in cardioprotection. To view the other articles in this section visit http://onlinelibrary.wiley.com/doi/10.1111/bph.v178.1/issuetoc.
Source: British Journal of Pharmacology
DOI: 10.1111/bph.15036
Language: English
Subfield: Pulmonary and Respiratory Medicine
Field: Medicine
Domain: Health Sciences
Open Access status: green
Awards: CardioPulmonary Vascular Biology COBRE, 3'UTR Shortening in Pulmonary Vascular Disease, Short-Term Training Program to Increase Diversity in Health-Related Research, Targeting CHI3L1 and its receptors in Hermansky-Pudlak Syndrome-associated lung disease, Sineoculis Homeobox Homolog 1 (Six1) in Pulmonary Fibrosis
OpenAlex ID: W3010552499