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Review
. 2021 Aug 27;22(17):9316.
doi: 10.3390/ijms22179316.

Extracellular Heat Shock Proteins as Therapeutic Targets and Biomarkers in Fibrosing Interstitial Lung Diseases

Julie Tanguy  1   2 , Lenny Pommerolle  1   2 , Carmen Garrido  1 , Martin Kolb  3 , Philippe Bonniaud  1   2   4 , Françoise Goirand  1   2   4 , Pierre-Simon Bellaye  2   5
Affiliations
Review

Extracellular Heat Shock Proteins as Therapeutic Targets and Biomarkers in Fibrosing Interstitial Lung Diseases

Julie Tanguy et al. Int J Mol Sci. .

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.

Keywords: IPF; biomarker; extracellular HSP; heat shock proteins; interstitial lung diseases; lung fibrosis.

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Conflict of interest statement

The authors declare no conflict of interest.

Figures

Figure 1
Figure 1
Schematic representation of HSPs’ biological functions. All HSPs mentioned are found in both intracellular and extracellular compartments.
Figure 2
Figure 2
eHSPs are a promising biomarker and therapeutic target in ILDs. IPF: idiopathic pulmonary fibrosis; NSIP: nonspecific interstitial pneumonia; SSc: systemic sclerosis; RA: rheumatoid arthritis; SA: sarcoidosis; PNC: pneumoconiosis; HP: hypersensitivity pneumonitis; DILD: drug-induced lung disease.
See this image and copyright information in PMC

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