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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MDPI AG
2021-06-01
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| Series: | International Journal of Molecular Sciences |
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| Online Access: | https://www.mdpi.com/1422-0067/22/12/6255 |
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| author | Anna Stainer Paola Faverio Sara Busnelli Martina Catalano Matteo Della Zoppa Almerico Marruchella Alberto Pesci Fabrizio Luppi |
| author_facet | Anna Stainer Paola Faverio Sara Busnelli Martina Catalano Matteo Della Zoppa Almerico Marruchella Alberto Pesci Fabrizio Luppi |
| author_sort | Anna Stainer |
| collection | DOAJ |
| 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. |
| first_indexed | 2024-03-10T10:31:18Z |
| format | Article |
| id | doaj.art-709264bd162140a4bc7ff21b1a22b45e |
| institution | Directory Open Access Journal |
| issn | 1661-6596 1422-0067 |
| language | English |
| last_indexed | 2024-03-10T10:31:18Z |
| publishDate | 2021-06-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | International Journal of Molecular Sciences |
| spelling | doaj.art-709264bd162140a4bc7ff21b1a22b45e2023-11-21T23:35:42ZengMDPI AGInternational Journal of Molecular Sciences1661-65961422-00672021-06-012212625510.3390/ijms22126255Molecular Biomarkers in Idiopathic Pulmonary Fibrosis: State of the Art and Future DirectionsAnna Stainer0Paola Faverio1Sara Busnelli2Martina Catalano3Matteo Della Zoppa4Almerico Marruchella5Alberto Pesci6Fabrizio Luppi7Department of Medicine and Surgery, University of Milano Bicocca, 20126 Milano, ItalyDepartment of Medicine and Surgery, University of Milano Bicocca, 20126 Milano, ItalyRespiratory Unit, San Gerardo Hospital, 20900 Monza, ItalyDepartment of Medicine and Surgery, University of Milano Bicocca, 20126 Milano, ItalyPulmonology Unit, Fondazione IRCCS Policlinico San Matteo, 27100 Pavia, ItalyRespiratory Unit, San Gerardo Hospital, 20900 Monza, ItalyDepartment of Medicine and Surgery, University of Milano Bicocca, 20126 Milano, ItalyDepartment of Medicine and Surgery, University of Milano Bicocca, 20126 Milano, ItalyIdiopathic 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.https://www.mdpi.com/1422-0067/22/12/6255idiopathic pulmonary fibrosisbiomarkerdiagnosisprediction |
| spellingShingle | Anna Stainer Paola Faverio Sara Busnelli Martina Catalano Matteo Della Zoppa Almerico Marruchella Alberto Pesci Fabrizio Luppi Molecular Biomarkers in Idiopathic Pulmonary Fibrosis: State of the Art and Future Directions International Journal of Molecular Sciences idiopathic pulmonary fibrosis biomarker diagnosis prediction |
| 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 | idiopathic pulmonary fibrosis biomarker diagnosis prediction |
| url | https://www.mdpi.com/1422-0067/22/12/6255 |
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