Treatment of pulmonary hypertension in idiopathic pulmonary fibrosis: shortfall in efficacy or trial design?

Abstract

Idiopathic pulmonary fibrosis (IPF) is a disease that carries a high mortality. Pulmonary hypertension (PH) frequently complicates the course of patients with IPF and is associated with significantly worse outcomes. Whether PH is a surrogate or driver of these worse outcomes remains unanswered, but the presence of PH represents an attractive target for therapy. This review delves into the various pulmonary vasoactive agents that have been subjected to study in IPF, the pitfalls of some of these prior studies, and attempts to lay a foundation for future study designs targeting PH in IPF.

Keywords:

• phenotype
• interstitial lung disease

Disclosure

Dr Nathan has received research funding and/or has consulted for Actelion, Boerhinger-Ingelheim, Bayer Pharmaceuticals, Gilead Sciences, Intermune, Roche, and United Therapeutics on the subjects of IPF and pulmonary hypertension. The authors report no other conflicts of interest in this work.