Current concepts of idiopathic pulmonary fibrosis: focus on biomarkers
https://doi.org/10.18093/0869-0189-2017-27-1-56-64
Abstract
Idiopathic pulmonary fibrosis (IPF) is a chronic progressive lung disease with a poor prognosis. In recent years, the development of international diagnostic criteria based on clinical, physiological, radiological, and histopathological appearances, improved diagnosis of IPF. However, currently available clinical data do not accurately predict the course of the disease, which can vary from a slowly progressing to rapidly progressive, and, in 5% of cases, is punctuated by episodes of rapid acute exacerbation. These challenges highlight the need for the development and validation of diagnostic biomarkers specific to IPF and prognostic biomarkers of future disease behavior to guide treatment decisions, including referral for transplant. The recent approval of pirfenidone and nintedanib and the identification of new potential therapeutic targets have created an urgent need for theragnostic markers, i.e. biomarkers able to assess, ideally at an early stage, therapeutic response to a given drug. This will avoid the side effects and increase efficacy of treatment. In addition, the currently available methods are not able to identify the IPF in the early stage to predict the course of disease, and to assess response to antifibrotic therapy. Recent advances in understanding the multiple interrelated pathogenic pathways underlying IPF have identified various molecular phenotypes resulting from complex interactions among genetic, epigenetic, transcriptional, post-transcriptional, metabolic, and environmental factors. The development and validation of diagnostic and prognostic biomarkers are necessary to enable a more precise and earlier diagnosis of IPF and to improve prediction of future disease behavior.
About the Author
E. Kh. Anaev
Federal Pulmonology Research Institute, Federal Medical and Biological Agency of Russia
Russian Federation
Doctor of Medicine, head of Laboratory of Non-invasive Diagnostic Methods, Clinical Division
Russian Federation
Doctor of Medicine, head of Laboratory of Non-invasive Diagnostic Methods, Clinical Division
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Anaev E.Kh. Current concepts of idiopathic pulmonary fibrosis: focus on biomarkers. PULMONOLOGIYA. 2017;27(1):56-64. (In Russ.) https://doi.org/10.18093/0869-0189-2017-27-1-56-64
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