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Molecular Biomarkers in Idiopathic Pulmonary Fibrosis: State of the Art and Future Directions
Idiopathic pulmonary fibrosis (IPF), the most lethal form of interstitial pneumonia of unknown cause, is associated with a specific radiological and histopathological pattern (the so-called “usual interstitial pneumonia” pattern) and has a median survival estimated to be between 3 and 5 years after...
Autores principales: | , , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
MDPI
2021
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8230407/ https://www.ncbi.nlm.nih.gov/pubmed/34200784 http://dx.doi.org/10.3390/ijms22126255 |
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author | Stainer, Anna Faverio, Paola Busnelli, Sara Catalano, Martina Della Zoppa, Matteo Marruchella, Almerico Pesci, Alberto Luppi, Fabrizio |
author_facet | Stainer, Anna Faverio, Paola Busnelli, Sara Catalano, Martina Della Zoppa, Matteo Marruchella, Almerico Pesci, Alberto Luppi, Fabrizio |
author_sort | Stainer, Anna |
collection | PubMed |
description | Idiopathic pulmonary fibrosis (IPF), the most lethal form of interstitial pneumonia of unknown cause, is associated with a specific radiological and histopathological pattern (the so-called “usual interstitial pneumonia” pattern) and has a median survival estimated to be between 3 and 5 years after diagnosis. However, evidence shows that IPF has different clinical phenotypes, which are characterized by a variable disease course over time. At present, the natural history of IPF is unpredictable for individual patients, although some genetic factors and circulating biomarkers have been associated with different prognoses. Since in its early stages, IPF may be asymptomatic, leading to a delayed diagnosis. Two drugs, pirfenidone and nintedanib, have been shown to modify the disease course by slowing down the decline in lung function. It is also known that 5–10% of the IPF patients may be affected by episodes of acute and often fatal decline. The acute worsening of disease is sometimes attributed to identifiable conditions, such as pneumonia or heart failure; but many of these events occur without an identifiable cause. These idiopathic acute worsenings are termed acute exacerbations of IPF. To date, clinical biomarkers, diagnostic, prognostic, and theranostic, are not well characterized. However, they could become useful tools helping facilitate diagnoses, monitoring disease progression and treatment efficacy. The aim of this review is to cover molecular mechanisms underlying IPF and research into new clinical biomarkers, to be utilized in diagnosis and prognosis, even in patients treated with antifibrotic drugs. |
format | Online Article Text |
id | pubmed-8230407 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2021 |
publisher | MDPI |
record_format | MEDLINE/PubMed |
spelling | pubmed-82304072021-06-26 Molecular Biomarkers in Idiopathic Pulmonary Fibrosis: State of the Art and Future Directions Stainer, Anna Faverio, Paola Busnelli, Sara Catalano, Martina Della Zoppa, Matteo Marruchella, Almerico Pesci, Alberto Luppi, Fabrizio Int J Mol Sci Review Idiopathic pulmonary fibrosis (IPF), the most lethal form of interstitial pneumonia of unknown cause, is associated with a specific radiological and histopathological pattern (the so-called “usual interstitial pneumonia” pattern) and has a median survival estimated to be between 3 and 5 years after diagnosis. However, evidence shows that IPF has different clinical phenotypes, which are characterized by a variable disease course over time. At present, the natural history of IPF is unpredictable for individual patients, although some genetic factors and circulating biomarkers have been associated with different prognoses. Since in its early stages, IPF may be asymptomatic, leading to a delayed diagnosis. Two drugs, pirfenidone and nintedanib, have been shown to modify the disease course by slowing down the decline in lung function. It is also known that 5–10% of the IPF patients may be affected by episodes of acute and often fatal decline. The acute worsening of disease is sometimes attributed to identifiable conditions, such as pneumonia or heart failure; but many of these events occur without an identifiable cause. These idiopathic acute worsenings are termed acute exacerbations of IPF. To date, clinical biomarkers, diagnostic, prognostic, and theranostic, are not well characterized. However, they could become useful tools helping facilitate diagnoses, monitoring disease progression and treatment efficacy. The aim of this review is to cover molecular mechanisms underlying IPF and research into new clinical biomarkers, to be utilized in diagnosis and prognosis, even in patients treated with antifibrotic drugs. MDPI 2021-06-10 /pmc/articles/PMC8230407/ /pubmed/34200784 http://dx.doi.org/10.3390/ijms22126255 Text en © 2021 by the authors. https://creativecommons.org/licenses/by/4.0/Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (https://creativecommons.org/licenses/by/4.0/). |
spellingShingle | Review Stainer, Anna Faverio, Paola Busnelli, Sara Catalano, Martina Della Zoppa, Matteo Marruchella, Almerico Pesci, Alberto Luppi, Fabrizio Molecular Biomarkers in Idiopathic Pulmonary Fibrosis: State of the Art and Future Directions |
title | Molecular Biomarkers in Idiopathic Pulmonary Fibrosis: State of the Art and Future Directions |
title_full | Molecular Biomarkers in Idiopathic Pulmonary Fibrosis: State of the Art and Future Directions |
title_fullStr | Molecular Biomarkers in Idiopathic Pulmonary Fibrosis: State of the Art and Future Directions |
title_full_unstemmed | Molecular Biomarkers in Idiopathic Pulmonary Fibrosis: State of the Art and Future Directions |
title_short | Molecular Biomarkers in Idiopathic Pulmonary Fibrosis: State of the Art and Future Directions |
title_sort | molecular biomarkers in idiopathic pulmonary fibrosis: state of the art and future directions |
topic | Review |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8230407/ https://www.ncbi.nlm.nih.gov/pubmed/34200784 http://dx.doi.org/10.3390/ijms22126255 |
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