Affiliation:
1. Pavlov First Saint Petersburg State Medical University
Abstract
A high proportion of patients with various interstitial lung diseases (ILD) develop progressive pulmonary fibrosis (PLF) associated with a deterioration in the quality of life, a decrease in functional status and early mortality. PLF is diagnosed in patients with ILD of known or unknown etiology other than idiopathic pulmonary fibrosis (ILF), who have radiological signs of pulmonary fibrosis, and at least two of the three criteria for progression, including clinical, functional and radiological indicators that have arisen over the past year in the absence of alternative causes of deterioration. Subtypes of ILD that are at risk of developing a progressive fibrosing phenotype include ILD associated with connective tissue diseases, such as rheumatoid arthritis and systemic scleroderma; sarcoidosis; hypersensitive pneumonitis, occupational lung diseases. In 2022, new criteria for the diagnosis of PLF appeared. The existing evidence of common pathogenesis mechanisms leading to progressive pulmonary fibrosis suggests the presence of uniform biomarkers of disease activity for a wide range of diseases. Pulmonary fibrosis is initiated by microinjuries of the alveolar epithelium of various etiologies, in the future its progression includes similar pathophysiological mechanisms, which suggests the presence of uniform biomarkers of disease activity for a wide range of diseases. Biomarkers can help not only identify patients at risk of progression, but also allow monitoring the early response to treatment. Biomarker research to date has identified a number of molecular markers that predict the presence of the disease, prognosis and/or response to treatment. The creation of national and international registries allows for long-term monitoring of patients with PLF, to answer questions about the long-term effectiveness of treatment. To date, a number of molecular factors that claim to be predictors are being discussed, allowing to verify the diagnosis, determine the prognosis and response to therapy.
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