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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Veröffentlicht in:International journal of molecular sciences Jg. 22; H. 12; S. 6255
Hauptverfasser: Stainer, Anna, Faverio, Paola, Busnelli, Sara, Catalano, Martina, Della Zoppa, Matteo, Marruchella, Almerico, Pesci, Alberto, Luppi, Fabrizio
Format: Journal Article
Sprache:Englisch
Veröffentlicht: Basel MDPI AG 10.06.2021
MDPI
Schlagworte:
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
Online-Zugang:Volltext
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Zusammenfassung: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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ISSN:1422-0067
1661-6596
1422-0067
DOI:10.3390/ijms22126255