Abstract
Interstitial lung diseases (ILDs) include a large number of diseases and causes with variable outcomes often associated with progressive fibrosis. Although each of the individual fibrosing ILDs are rare, collectively, they affect a considerable number of patients, representing a significant burden of disease. Idiopathic pulmonary fibrosis (IPF) is the typical chronic fibrosing ILD associated with progressive decline in lung. Other fibrosing ILDs are often associated with connective tissues diseases, including rheumatoid arthritis-ILD (RA-ILD) and systemic sclerosis-associated ILD (SSc-ILD), or environmental/drug exposure. Given the vast number of progressive fibrosing ILDs and the disparities in clinical patterns and disease features, the course of these diseases is heterogeneous and cannot accurately be predicted for an individual patient. As a consequence, the discovery of novel biomarkers for these types of diseases is a major clinical challenge. Heat shock proteins (HSPs) are molecular chaperons that have been extensively described to be involved in fibrogenesis. Their extracellular forms (eHSPs) have been recently and successfully used as therapeutic targets or circulating biomarkers in cancer. The current review will describe the role of eHSPs in fibrosing ILDs, highlighting the importance of these particular stress proteins to develop new therapeutic strategies and discover potential biomarkers in these diseases.
Highlights
Interstitial lung diseases (ILDs) is a group of respiratory lung diseases affecting the lung interstitium which may lead to progressive lung fibrosis and respiratory failure
This review highlights the role of extracellular Heat shock proteins (HSPs) and their potential use as diagnostic and therapeutic tools in fibrosing interstitial lung disease (Figure 2 and Table 2)
Despite the growing body of evidence demonstrating that HSPs are largely involved in fibrosing ILDs, the diagnostic or therapeutic strategies involving HSPs’ measurement or inhibition/upregulation are far from being investigated
Summary
Interstitial lung diseases (ILDs) is a group of respiratory lung diseases affecting the lung interstitium which may lead to progressive lung fibrosis and respiratory failure. PDGF is known to stimulate fibroblast proliferation and migration whereas IL-10 is an anti-inflammatory cytokine that promotes fibrosis by stimulating macrophage recruitment [7,8] Such a pro-fibrotic microenvironment leads to the activation of neighboring cells and drives the progression of fibrosis. HSPs take part in several molecular mechanisms thereby favoring oncogenesis, tumor progression and/or cancer cell resistance They inhibit key effectors of apoptosis machinery at the pre- and post-mitochondrial level, such as HSP27 associating cytochrome C, HSP90 inhibiting adaptor molecule apoptotic protease activation factor-1 (Apaf-1) or HSP70 associating Apaf-1 and apoptosis inducing factor (AIF). HSPs appeared to be beneficial for cancerous cells and thereby deleterious for patients affected by a wide range of cancer types Their depletion induces the regression of the tumors [14].
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