Targeting Progression in Pulmonary Fibrosis: An Overview of Underlying Mechanisms, Molecular Biomarkers, and Therapeutic Intervention
Interstitial lung diseases comprise a heterogenous range of diffuse lung disorders, potentially resulting in pulmonary fibrosis. While idiopathic pulmonary fibrosis has been recognized as the paradigm of a progressive fibrosing interstitial lung disease, other conditions with a progressive fibrosing...
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MDPI AG
2024-02-01
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| Series: | Life |
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| Online Access: | https://www.mdpi.com/2075-1729/14/2/229 |
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| author | Vito D’Agnano Domenica Francesca Mariniello Michela Ruotolo Gianluca Quarcio Alessandro Moriello Stefano Conte Antonio Sorrentino Stefano Sanduzzi Zamparelli Andrea Bianco Fabio Perrotta |
| author_facet | Vito D’Agnano Domenica Francesca Mariniello Michela Ruotolo Gianluca Quarcio Alessandro Moriello Stefano Conte Antonio Sorrentino Stefano Sanduzzi Zamparelli Andrea Bianco Fabio Perrotta |
| author_sort | Vito D’Agnano |
| collection | DOAJ |
| description | Interstitial lung diseases comprise a heterogenous range of diffuse lung disorders, potentially resulting in pulmonary fibrosis. While idiopathic pulmonary fibrosis has been recognized as the paradigm of a progressive fibrosing interstitial lung disease, other conditions with a progressive fibrosing phenotype characterized by a significant deterioration of the lung function may lead to a burden of significant symptoms, a reduced quality of life, and increased mortality, despite treatment. There is now evidence indicating that some common underlying biological mechanisms can be shared among different chronic fibrosing disorders; therefore, different biomarkers for disease-activity monitoring and prognostic assessment are under evaluation. Thus, understanding the common pathways that induce the progression of pulmonary fibrosis, comprehending the diversity of these diseases, and identifying new molecular markers and potential therapeutic targets remain highly crucial assignments. The purpose of this review is to examine the main pathological mechanisms regulating the progression of fibrosis in interstitial lung diseases and to provide an overview of potential biomarker and therapeutic options for patients with progressive pulmonary fibrosis. |
| first_indexed | 2024-03-07T22:24:03Z |
| format | Article |
| id | doaj.art-b9e3d524c0384c76a934442e53c12e62 |
| institution | Directory Open Access Journal |
| issn | 2075-1729 |
| language | English |
| last_indexed | 2024-03-07T22:24:03Z |
| publishDate | 2024-02-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | Life |
| spelling | doaj.art-b9e3d524c0384c76a934442e53c12e622024-02-23T15:24:42ZengMDPI AGLife2075-17292024-02-0114222910.3390/life14020229Targeting Progression in Pulmonary Fibrosis: An Overview of Underlying Mechanisms, Molecular Biomarkers, and Therapeutic InterventionVito D’Agnano0Domenica Francesca Mariniello1Michela Ruotolo2Gianluca Quarcio3Alessandro Moriello4Stefano Conte5Antonio Sorrentino6Stefano Sanduzzi Zamparelli7Andrea Bianco8Fabio Perrotta9Department of Translational Medical Sciences, University of Campania L. Vanvitelli, 80131 Naples, ItalyDepartment of Translational Medical Sciences, University of Campania L. Vanvitelli, 80131 Naples, ItalyDepartment of Translational Medical Sciences, University of Campania L. Vanvitelli, 80131 Naples, ItalyDepartment of Translational Medical Sciences, University of Campania L. Vanvitelli, 80131 Naples, ItalyDepartment of Translational Medical Sciences, University of Campania L. Vanvitelli, 80131 Naples, ItalyDepartment of Translational Medical Sciences, University of Campania L. Vanvitelli, 80131 Naples, ItalyDepartment of Translational Medical Sciences, University of Campania L. Vanvitelli, 80131 Naples, ItalyDivision of Pneumology, A. Cardarelli Hospital, 80131 Naples, ItalyDepartment of Translational Medical Sciences, University of Campania L. Vanvitelli, 80131 Naples, ItalyDepartment of Translational Medical Sciences, University of Campania L. Vanvitelli, 80131 Naples, ItalyInterstitial lung diseases comprise a heterogenous range of diffuse lung disorders, potentially resulting in pulmonary fibrosis. While idiopathic pulmonary fibrosis has been recognized as the paradigm of a progressive fibrosing interstitial lung disease, other conditions with a progressive fibrosing phenotype characterized by a significant deterioration of the lung function may lead to a burden of significant symptoms, a reduced quality of life, and increased mortality, despite treatment. There is now evidence indicating that some common underlying biological mechanisms can be shared among different chronic fibrosing disorders; therefore, different biomarkers for disease-activity monitoring and prognostic assessment are under evaluation. Thus, understanding the common pathways that induce the progression of pulmonary fibrosis, comprehending the diversity of these diseases, and identifying new molecular markers and potential therapeutic targets remain highly crucial assignments. The purpose of this review is to examine the main pathological mechanisms regulating the progression of fibrosis in interstitial lung diseases and to provide an overview of potential biomarker and therapeutic options for patients with progressive pulmonary fibrosis.https://www.mdpi.com/2075-1729/14/2/229progressive pulmonary fibrosisbiomarkersantifibroticsnintedanibpirfenidone |
| spellingShingle | Vito D’Agnano Domenica Francesca Mariniello Michela Ruotolo Gianluca Quarcio Alessandro Moriello Stefano Conte Antonio Sorrentino Stefano Sanduzzi Zamparelli Andrea Bianco Fabio Perrotta Targeting Progression in Pulmonary Fibrosis: An Overview of Underlying Mechanisms, Molecular Biomarkers, and Therapeutic Intervention Life progressive pulmonary fibrosis biomarkers antifibrotics nintedanib pirfenidone |
| title | Targeting Progression in Pulmonary Fibrosis: An Overview of Underlying Mechanisms, Molecular Biomarkers, and Therapeutic Intervention |
| title_full | Targeting Progression in Pulmonary Fibrosis: An Overview of Underlying Mechanisms, Molecular Biomarkers, and Therapeutic Intervention |
| title_fullStr | Targeting Progression in Pulmonary Fibrosis: An Overview of Underlying Mechanisms, Molecular Biomarkers, and Therapeutic Intervention |
| title_full_unstemmed | Targeting Progression in Pulmonary Fibrosis: An Overview of Underlying Mechanisms, Molecular Biomarkers, and Therapeutic Intervention |
| title_short | Targeting Progression in Pulmonary Fibrosis: An Overview of Underlying Mechanisms, Molecular Biomarkers, and Therapeutic Intervention |
| title_sort | targeting progression in pulmonary fibrosis an overview of underlying mechanisms molecular biomarkers and therapeutic intervention |
| topic | progressive pulmonary fibrosis biomarkers antifibrotics nintedanib pirfenidone |
| url | https://www.mdpi.com/2075-1729/14/2/229 |
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