Abstract
Interstitial lung disease (ILD) comprises a heterogenous group of diffuse lung disorders that commonly result in irreversible pulmonary fibrosis. While idiopathic pulmonary fibrosis (IPF) is the prototypical progressive fibrosing ILD (PF-ILD), a high proportion of patients with other ILD subtypes develop a PF-ILD phenotype. Evidence exists for shared pathobiology leading to progressive fibrosis, suggesting that biomarkers of disease activity may prove informative across the wide spectrum of ILDs. Biomarker investigation to date has identified a number of molecular markers that predict relevant ILD endpoints, including disease presence, prognosis, and/or treatment response. In this review, we provide an overview of potentially informative biomarkers in patients with ILD, including those suggestive of a PF-ILD phenotype. We highlight the recent genomic, transcriptomic, and proteomic investigations that identified these biomarkers and discuss the body compartments in which they are found, including the peripheral blood, airway, and lung parenchyma. Finally, we identify critical gaps in knowledge within the field of ILD biomarker research and propose steps to advance the field toward biomarker implementation.
Highlights
Interstitial lung disease (ILD) comprises a heterogeneous group of diffuse parenchymal lung processes characterized by variable degrees of inflammation and fibrosis
Bronchoalveolar lavage (BAL) protein biomarkers, including YKL-40, IL-15, IL-2, and TNF have been linked to differential survival in various ILD subtypes [101, 150, 192], but these studies have been generally limited by small sample sizes
Because usual interstitial pneumonia (UIP) can be observed in other ILD subtypes, including RAILD, SSc-ILD, asbestosis, and chronic hypersensitivity pneumonitis (CHP) [2, 199,200,201,202], it remains unclear how well this molecular diagnostic tool discriminates UIP due to idiopathic pulmonary fibrosis (IPF) from UIP seen in these other conditions
Summary
Interstitial lung disease (ILD) comprises a heterogeneous group of diffuse parenchymal lung processes characterized by variable degrees of inflammation and fibrosis. Biomarkers have advanced our understanding of IPF and are beginning to inform diagnosis, prognosis, and treatment in patients with ILD, including those with PF-ILD. Sources of biomarkers that may inform diagnosis, outcomes, and treatment response in ILD include the peripheral blood, airway, and lung parenchyma.
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