INTERSTITIAL LUNG DISEASE: Edited by Francesco Bonella, Athol U. Wells and Justin OldhamPulmonary hypertension in interstitial lung disease: screening, diagnosis and treatmentBehr, Jürgena; Nathan, Steven D.b Author Information aDepartment 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 bAdvanced Lung Disease and Transplant Program, Inova Fairfax Hospital, Falls Church, Virginia, USA Correspondence to Jürgen Behr, Department of Medicine V, University Hospital, LMU Munich, Member of the German Center for Lung Research (DZL), Marchioninistr. 15, 81377 Munich, Germany. Tel: 49 89 44007 5310; e-mail: [email protected] Current Opinion in Pulmonary Medicine 27(5):p 396-404, September 2021. | DOI: 10.1097/MCP.0000000000000790 Buy Metrics 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. Copyright © 2021 Wolters Kluwer Health, Inc. All rights reserved.