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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...

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Autores principales: Stainer, Anna, Faverio, Paola, Busnelli, Sara, Catalano, Martina, Della Zoppa, Matteo, Marruchella, Almerico, Pesci, Alberto, Luppi, Fabrizio
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2021
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.
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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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