title: 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.
authors: Advanced Lung Disease Clinic, Inova Fairfax Hospital, Falls Church, VA, USA.
authors: Steven D
authors: SD
authors: Nathan
authors: Advanced Lung Disease Clinic, Inova Fairfax Hospital, Falls Church, VA, USA.
authors: Christopher S
authors: CS
authors: King
mesh_terms: D002986:Clinical Trials as Topic / Q000379:methods; D006801:Humans; D006976:Hypertension, Pulmonary / Q000188:drug therapy* / Q000209:etiology / Q000503:physiopathology; D054990:Idiopathic Pulmonary Fibrosis / Q000150:complications* / Q000188:drug therapy / Q000503:physiopathology; D017063:Outcome Assessment, Health Care; D012107:Research Design; D014665:Vasodilator Agents / Q000627:therapeutic use*
chemical_list: D014665:Vasodilator Agents
keywords: interstitial lung disease; phenotype