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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Published in:	International journal of molecular sciences Vol. 22; no. 12; p. 6255
Main Authors:	Stainer, Anna, Faverio, Paola, Busnelli, Sara, Catalano, Martina, Della Zoppa, Matteo, Marruchella, Almerico, Pesci, Alberto, Luppi, Fabrizio
Format:	Journal Article
Language:	English
Published:	Basel MDPI AG 10.06.2021 MDPI
Subjects:	Biomarkers Chronic obstructive pulmonary disease Disease prevention Extracellular matrix Medical prognosis Pathogenesis Polymorphism Proteins Pulmonary fibrosis Review Surfactants Telomerase
ISSN:	1422-0067, 1661-6596, 1422-0067
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Abstract	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.
AbstractList	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. 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.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.
Author	Faverio, Paola Busnelli, Sara Marruchella, Almerico Stainer, Anna Pesci, Alberto Della Zoppa, Matteo Luppi, Fabrizio Catalano, Martina
AuthorAffiliation	1 Department of Medicine and Surgery, University of Milano Bicocca, 20126 Milano, Italy; annetta.stainer@gmail.com (A.S.); paola.faverio@unimib.it (P.F.); m.catalano17@campus.unimib.it (M.C.); alberto.pesci@unimib.it (A.P.) 2 Respiratory Unit, San Gerardo Hospital, 20900 Monza, Italy; busnelli.sara@gmail.com (S.B.); a.marruchella@asst-monza.it (A.M.) 3 Pulmonology Unit, Fondazione IRCCS Policlinico San Matteo, 27100 Pavia, Italy; matteo.dellazoppa@gmail.com
AuthorAffiliation_xml	– name: 1 Department of Medicine and Surgery, University of Milano Bicocca, 20126 Milano, Italy; annetta.stainer@gmail.com (A.S.); paola.faverio@unimib.it (P.F.); m.catalano17@campus.unimib.it (M.C.); alberto.pesci@unimib.it (A.P.) – name: 3 Pulmonology Unit, Fondazione IRCCS Policlinico San Matteo, 27100 Pavia, Italy; matteo.dellazoppa@gmail.com – name: 2 Respiratory Unit, San Gerardo Hospital, 20900 Monza, Italy; busnelli.sara@gmail.com (S.B.); a.marruchella@asst-monza.it (A.M.)
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Snippet	Idiopathic pulmonary fibrosis (IPF), the most lethal form of interstitial pneumonia of unknown cause, is associated with a specific radiological and...
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SubjectTerms	Biomarkers Chronic obstructive pulmonary disease Disease prevention Extracellular matrix Medical prognosis Pathogenesis Polymorphism Proteins Pulmonary fibrosis Review Surfactants Telomerase
Title	Molecular Biomarkers in Idiopathic Pulmonary Fibrosis: State of the Art and Future Directions
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