真Check for updatesD0L:10.1002/pdl2.12213REVIEW ARTICLEPulmonary CirculationPV WILEYPathogenesis,clinical features,and phenotypes ofpulmonary hypertension associated with interstitial lungdisease:A consensus statement from the PulmonaryVascular Research Institute's Innovative DrugDevelopment Initiative-Group 3 Pulmonary HypertensionLucilla Piccari Brian Allwood2 Katerina Antoniou3Jonathan H.Chung Paul M.Hassouns Sylvia M.Nikkho5Rajan Saggar?Oksana A.Shlobins Patrizio VituloSteven D.Nathans Stephen John Wort10.11Department of Pulmonary Medicine,Hospital del Mar,Barcelona,SpainDepartment of Medicine,Division of Pulmonology,Stellenbosch University Tygerberg Hospital,Cape Town,South AfricaSDepartment of Thoracic Medicine,University of Crete School of Medicine,Heraklion,Crete,GreeceDepartment of Radiology,The University of Chicago Medicine,Chicago,Ilinois,USASDepartment of Medicine,Division of Pulmonary and Critical Care Medicine,Johns Hopkins University,Baltimore,Maryland,USA"Global Clinical Development,Bayer AG,Berlin,GermanyLung&Heart-Lung Transplant and Pulmonary Hypertension Programs,University of California Los Angeles David Geffen School of Medicine,LosAngeles,California,USAAdvanced Lung Disease and Transplant Program,Inova Health System,Falls Church,Virginia,USADepartment of Pulmonary Medicine,IRCCS Mediterranean Institute for Transplantation and Advanced Specialized Therapies,Palermo,Sicilia,ItalyNational Pulmonary Hypertension Service at the Royal Brompton Hospital,London,UKNational Heart and Lung Institute,Imperial College,London,UKCorrespondenceLucilla Piccari,Department of PulmonaryAbstractMedicine,Pulmonary Hypertension Unit,Pulmonary hypertension (PH)is a frequent complication of interstitial lungHospital del Mar,Passeig Maritim 25-27.Barcelona 08003,Spaindisease (ILD).Although PH has mostly been described in idiopathicEmail:lucilla.piccari@gmail.compulmonary fibrosis,it can manifest in association with many other forms ofILD.Associated pathogenetic mechanisms are complex and incompletelyFunding informationNoneunderstood but there is evidence of disruption of molecular and geneticpathways,with panvascular histopathologic changes,multiple patho-physiologic sequelae,and profound clinical ramifications.While there aresome recognized clinical phenotypes such as combined pulmonary fibrosisSteven D.Nathan and Stephen John Wort contributed equally to this work,therefore they are both last author.This is an open access article under the terms of the Creative Commons Attribution-NonCommercial License,which permits use,distribution and reproduction in anymedium,provided the original work is properly cited and is not used for commercial purposes.2023 The Authors.Pulmonary Circulation published by John Wiley Sons Ltd on behalf of Pulmonary Vascular Research Institute.Pulmonary Circulation.2023:13:e12213.wileyonlinelibrary.com/journal/pul2 1 of 21https://doi.org/10.1002/pul2.12213
