Vascular involvement in idiopathic pulmonary fibrosis
Background Idiopathic pulmonary fibrosis (IPF) is a chronic, fibrosing and progressive interstitial lung disease of unknown aetiology with a pathogenesis still partly unknown. Several microvascular and macrovascular abnormalities have been demonstrated in the pathogenesis of IPF and related pulmonar...
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| Format: | Article |
| Language: | English |
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European Respiratory Society
2024-11-01
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| Series: | ERJ Open Research |
| Online Access: | http://openres.ersjournals.com/content/10/6/00550-2024.full |
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| author | Michele Mondoni Rocco Rinaldo Christopher J. Ryerson Cristina Albrici Andrea Baccelli Claudio Tirelli Francesca Marchetti Jacopo Cefalo Giulia Nalesso Giulia Ferranti Fausta Alfano Giovanni Sotgiu Marco Guazzi Stefano Centanni |
| author_facet | Michele Mondoni Rocco Rinaldo Christopher J. Ryerson Cristina Albrici Andrea Baccelli Claudio Tirelli Francesca Marchetti Jacopo Cefalo Giulia Nalesso Giulia Ferranti Fausta Alfano Giovanni Sotgiu Marco Guazzi Stefano Centanni |
| author_sort | Michele Mondoni |
| collection | DOAJ |
| description | Background
Idiopathic pulmonary fibrosis (IPF) is a chronic, fibrosing and progressive interstitial lung disease of unknown aetiology with a pathogenesis still partly unknown. Several microvascular and macrovascular abnormalities have been demonstrated in the pathogenesis of IPF and related pulmonary hypertension (PH), a complication of the disease.
Methods
We carried out a non-systematic, narrative literature review aimed at describing the role of the vasculature in the natural history of IPF.
Results
The main molecular pathogenetic mechanisms involving vasculature (i.e. endothelial-to-mesenchymal transition, vascular remodelling, endothelial permeability, occult alveolar haemorrhage, vasoconstriction and hypoxia) and the genetic basis of vascular remodelling are described. The prevalence and clinical relevance of associated PH are highlighted with focus on the vasculature as a prognostic marker. The vascular effects of current antifibrotic therapies, the role of pulmonary vasodilators in the treatment of disease, and new pharmacological options with vascular-targeted activity are described.
Conclusions
The vasculature plays a key role in the natural history of IPF from the early phases of disease until development of PH in a subgroup of patients, a complication related to a worse prognosis. Pulmonary vascular volume has emerged as a novel computed tomography finding and a predictor of mortality, independent of PH. New pharmacological options with concomitant vascular-directed activity might be promising in the treatment of IPF. |
| format | Article |
| id | doaj-art-f7f58beeffc5467e93ce034f79b146de |
| institution | Kabale University |
| issn | 2312-0541 |
| language | English |
| publishDate | 2024-11-01 |
| publisher | European Respiratory Society |
| record_format | Article |
| series | ERJ Open Research |
| spelling | doaj-art-f7f58beeffc5467e93ce034f79b146de2025-01-14T09:50:21ZengEuropean Respiratory SocietyERJ Open Research2312-05412024-11-0110610.1183/23120541.00550-202400550-2024Vascular involvement in idiopathic pulmonary fibrosisMichele Mondoni0Rocco Rinaldo1Christopher J. Ryerson2Cristina Albrici3Andrea Baccelli4Claudio Tirelli5Francesca Marchetti6Jacopo Cefalo7Giulia Nalesso8Giulia Ferranti9Fausta Alfano10Giovanni Sotgiu11Marco Guazzi12Stefano Centanni13 Department of Health Sciences, Respiratory Unit, ASST Santi Paolo e Carlo, Università degli Studi di Milano, Milan, Italy Department of Medical Sciences, Respiratory Diseases Unit, AOU Città della Salute e della Scienza di Torino, Molinette Hospital, University of Turin, Turin, Italy Department of Medicine and Centre for Heart Lung Innovation, University of British Columbia, Vancouver, Canada Department of Health Sciences, Respiratory Unit, ASST Santi Paolo e Carlo, Università degli Studi di Milano, Milan, Italy Department of Respiratory Medicine, Royal Brompton Hospital, Guy's and St Thomas’ NHS Foundation Trust, London, UK Department of Health Sciences, Respiratory Unit, ASST Santi Paolo e Carlo, Università degli Studi di Milano, Milan, Italy Department of Health Sciences, Respiratory Unit, ASST Santi Paolo e Carlo, Università degli Studi di Milano, Milan, Italy Department of Health Sciences, Respiratory Unit, ASST Santi Paolo e Carlo, Università degli Studi di Milano, Milan, Italy Department of Health Sciences, Respiratory Unit, ASST Santi Paolo e Carlo, Università degli Studi di Milano, Milan, Italy Department of Health Sciences, Respiratory Unit, ASST Santi Paolo e Carlo, Università degli Studi di Milano, Milan, Italy Department of Health Sciences, Respiratory Unit, ASST Santi Paolo e Carlo, Università degli Studi di Milano, Milan, Italy Dept of Medical, Clinical Epidemiology and Medical Statistics Unit, Surgical and Experimental Sciences, University of Sassari, Sassari, Italy Department of Cardiology, University of Milano School of Medicine, San Paolo Hospital, ASST Santi Paolo e Carlo, Milan, Italy Department of Health Sciences, Respiratory Unit, ASST Santi Paolo e Carlo, Università degli Studi di Milano, Milan, Italy Background Idiopathic pulmonary fibrosis (IPF) is a chronic, fibrosing and progressive interstitial lung disease of unknown aetiology with a pathogenesis still partly unknown. Several microvascular and macrovascular abnormalities have been demonstrated in the pathogenesis of IPF and related pulmonary hypertension (PH), a complication of the disease. Methods We carried out a non-systematic, narrative literature review aimed at describing the role of the vasculature in the natural history of IPF. Results The main molecular pathogenetic mechanisms involving vasculature (i.e. endothelial-to-mesenchymal transition, vascular remodelling, endothelial permeability, occult alveolar haemorrhage, vasoconstriction and hypoxia) and the genetic basis of vascular remodelling are described. The prevalence and clinical relevance of associated PH are highlighted with focus on the vasculature as a prognostic marker. The vascular effects of current antifibrotic therapies, the role of pulmonary vasodilators in the treatment of disease, and new pharmacological options with vascular-targeted activity are described. Conclusions The vasculature plays a key role in the natural history of IPF from the early phases of disease until development of PH in a subgroup of patients, a complication related to a worse prognosis. Pulmonary vascular volume has emerged as a novel computed tomography finding and a predictor of mortality, independent of PH. New pharmacological options with concomitant vascular-directed activity might be promising in the treatment of IPF.http://openres.ersjournals.com/content/10/6/00550-2024.full |
| spellingShingle | Michele Mondoni Rocco Rinaldo Christopher J. Ryerson Cristina Albrici Andrea Baccelli Claudio Tirelli Francesca Marchetti Jacopo Cefalo Giulia Nalesso Giulia Ferranti Fausta Alfano Giovanni Sotgiu Marco Guazzi Stefano Centanni Vascular involvement in idiopathic pulmonary fibrosis ERJ Open Research |
| title | Vascular involvement in idiopathic pulmonary fibrosis |
| title_full | Vascular involvement in idiopathic pulmonary fibrosis |
| title_fullStr | Vascular involvement in idiopathic pulmonary fibrosis |
| title_full_unstemmed | Vascular involvement in idiopathic pulmonary fibrosis |
| title_short | Vascular involvement in idiopathic pulmonary fibrosis |
| title_sort | vascular involvement in idiopathic pulmonary fibrosis |
| url | http://openres.ersjournals.com/content/10/6/00550-2024.full |
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