Idiopathic pulmonary fibrosis and pulmonary hypertension: Heracles meets the Hydra

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.

Automated summary

IPF is a fatal lung disease that also leads to pulmonary hypertension, reducing survival rates. Patients with both conditions have worse outcomes compared to those with only one, necessitating the development of improved treatments for PH in the setting of IPF.