Bowman Willis S, Echt Gabrielle A, Oldham Justin M
Division of Pulmonary, Critical Care, and Sleep Medicine, University of California, Davis, Davis, CA, United States.
Front Med (Lausanne). 2021 May 10;8:680997. doi: 10.3389/fmed.2021.680997. eCollection 2021.
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.
间质性肺疾病（ILD）是一组异质性的弥漫性肺部疾病，通常会导致不可逆的肺纤维化。虽然特发性肺纤维化（IPF）是典型的进行性纤维化ILD（PF-ILD），但很大一部分其他ILD亚型的患者会发展为PF-ILD表型。有证据表明存在导致进行性纤维化的共同病理生物学机制，这表明疾病活动的生物标志物可能对广泛的ILD具有指导意义。迄今为止的生物标志物研究已经确定了一些预测相关ILD终点的分子标志物，包括疾病的存在、预后和/或治疗反应。在本综述中，我们概述了ILD患者中可能具有指导意义的生物标志物，包括那些提示PF-ILD表型的生物标志物。我们重点介绍了最近确定这些生物标志物的基因组、转录组和蛋白质组学研究，并讨论了发现它们的身体部位，包括外周血、气道和肺实质。最后，我们确定了ILD生物标志物研究领域的关键知识空白，并提出了推动该领域实现生物标志物应用的步骤。
