Lucilla Piccari, Brian Allwood, Katerina Antoniou, Jonathan H. Chung, Paul M. Hassoun, Sylvia M. Nikkho, Rajan Saggar, Oksana A. Shlobin, Patrizio Vitulo, Steven D. Nathan, Stephen John Wort
Hospital del Mar. Stellenbosch University and Tygerberg Hospital. University of Crete School of Medicine. The University of Chicago. Johns Hopkins University. Bayer AG. University of California Los Angeles David Geffen School of Medicine. Inova Health System. IRCCS Mediterranean Institute for Transplantation and Advanced Specialized Therapies. Royal Brompton Hospital. Imperial College.
Spain, South Africa, Greece, United States, Germany, Italy and United Kingdom.
Pulmonary Circulation
Pulm Circ 2023;
DOI: 10.1002/pul2.12213
Abstract
Pulmonary hypertension (PH) is a frequent complication of interstitial lung disease (ILD). Although PH has mostly been described in idiopathic pulmonary fibrosis, it can manifest in association with many other forms of ILD. Associated pathogenetic mechanisms are complex and incompletely understood but there is evidence of disruption of molecular and genetic pathways, with panvascular histopathologic changes, multiple pathophysiologic sequelae, and profound clinical ramifications. While there are some recognized clinical phenotypes such as combined pulmonary fibrosis and emphysema and some possible phenotypes such as connective tissue disease associated with ILD and PH, the identification of further phenotypes of PH in ILD has thus far proven elusive. This statement reviews the current evidence on the pathogenesis, recognized patterns, and useful diagnostic tools to detect phenotypes of PH in ILD. Distinct phenotypes warrant recognition if they are characterized through either a distinct presentation, clinical course, or treatment response. Furthermore, we propose a set of recommendations for future studies that might enable the recognition of new phenotypes.
Category
Class III. Pulmonary Hypertension Associated with Lung Disease
Review Articles Concerning Pulmonary Vascular Disease
Age Focus: Adult Pulmonary Vascular Disease
Fresh or Filed Publication: Filed (PHiled). Greater than 1-2 years since publication
Article Access
Free PDF File or Full Text Article Available Through PubMed or DOI: Yes