Jürgen Behr1, Steven D Nathan. 1. Department of Medicine V, University Hospital, LMU Munich and Lungenforschungsambulanz Helmholtz Zentrum Munich, Comprehensive Pneumology Center, Member of the German Center for Lung Research, Munich, Germany Advanced Lung Disease and Transplant Program, Inova Fairfax Hospital, Falls Church, Virginia, USA.
Abstract
PURPOSE OF REVIEW: Pulmonary vascular disease resulting in pulmonary hypertension in the context of interstitial lung disease (PH-ILD) is a common complication that presents many challenges in clinical practice. Despite recent advances, the pathogenetic interplay between parenchymal and vascular disease in ILD is not fully understood. This review provides an overview of the current knowledge and recent advances in the field. RECENT FINDINGS: Clinical trials employing the phosphodiesterase-5-inhibitor sildenafil delivered negative results whereas riociguat showed harmful effects in the PH-ILD population. More recently, inhaled treprostinil showed positive effects on the primary endpoint (six-min walk-distance) in the largest prospective randomized placebo-controlled trial to date in this patient population. Additionally, a pilot trial of ambulatory inhaled nitric oxide suggests beneficial effects based on the novel endpoint of actigraphy. SUMMARY: In view of these novel developments this review provides an overview of the status quo of screening, diagnosis and management of pulmonary vascular disease and PH in patients with ILD.
PURPOSE OF REVIEW: Pulmonary vascular disease resulting in pulmonary hypertension in the context of interstitial lung disease (PH-ILD) is a common complication that presents many challenges in clinical practice. Despite recent advances, the pathogenetic interplay between parenchymal and vascular disease in ILD is not fully understood. This review provides an overview of the current knowledge and recent advances in the field. RECENT FINDINGS: Clinical trials employing the phosphodiesterase-5-inhibitor sildenafil delivered negative results whereas riociguat showed harmful effects in the PH-ILD population. More recently, inhaled treprostinil showed positive effects on the primary endpoint (six-min walk-distance) in the largest prospective randomized placebo-controlled trial to date in this patient population. Additionally, a pilot trial of ambulatory inhaled nitric oxide suggests beneficial effects based on the novel endpoint of actigraphy. SUMMARY: In view of these novel developments this review provides an overview of the status quo of screening, diagnosis and management of pulmonary vascular disease and PH in patients with ILD.
Authors: Christopher A Thomas; Justin Lee; Roberto J Bernardo; Ryan J Anderson; Vladimir Glinskii; Yon K Sung; Kristina Kudelko; Haley Hedlin; Andrew Sweatt; Steven M Kawut; Rishi Raj; Roham T Zamanian; Vinicio de Jesus Perez Journal: Front Med (Lausanne) Date: 2021-12-03