TY - JOUR
T1 - Idiopathic pulmonary fibrosis and pulmonary hypertension
T2 - Heracles meets the Hydra
AU - Rajagopal, Keshava
AU - Bryant, Andrew J.
AU - Sahay, Sandeep
AU - Wareing, Nancy
AU - Zhou, Yang
AU - Pandit, Lavannya M.
AU - Karmouty-Quintana, Harry
N1 - © 2020 The British Pharmacological Society.
PY - 2021/1
Y1 - 2021/1
N2 - 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.
AB - 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.
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U2 - 10.1111/bph.15036
DO - 10.1111/bph.15036
M3 - Review article
C2 - 32128790
AN - SCOPUS:85083106937
SN - 0007-1188
VL - 178
SP - 172
EP - 186
JO - British Journal of Pharmacology
JF - British Journal of Pharmacology
IS - 1
ER -