Immune mechanisms and novel therapies for idiopathic pulmonary fibrosis

Abstract

Idiopathic pulmonary fibrosis (IPF), a progressive lung disease characterized by irreversible lung dysfunction caused by fibroblast proliferation and excessive collagen deposition, is the result of persistent chronic inflammation of the lung parenchyma. Although the pathogenesis is not fully understood, the role of immune mechanisms such as innate immune response, adaptive immunity and immune regulation, and cytokines in the pathophysiological mechanism of pulmonary fibrosis have been gradually recognized. There are currently limited drugs available to treat IPF, and long-term use of these drugs may have many adverse effects. With the elucidation of the underlying immunological pathogenesis, the development of more valuable drugs targeting the immune system becomes possible. This review introduces the immunological pathogenesis of pulmonary fibrosis and the emerging drugs targeting the immune system in recent years, aiming to provide insights into the mechanism and treatment direction of pulmonary fibrosis.

Keywords

Pulmonary fibrosis / Immune mechanisms / Novel treatments / Therapeutic targets

CRediT authorship contribution statement

Zhiguang Zhai and Wenxiang Zhu conceived and designed the work. Dongyang Gao and Wenli Gao were responsible for writing. Wenxiang Zhu was in charge of checking and revision. All the figures in the article were made by Dongyang Gao. All the authors have read and approved the final manuscript.

Data availability

No data was used for the research described in the article.

Ethics approval

Not applicable.

Funding information

This study was supported by the Basic Research Project of the Science, Technology, and Innovation Commission of Shenzhen Municipality (JCYJ20210324135410028).

Declaration of competing interest

The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper.

Acknowledgments

All the authors thank Miss Dou for her contribution in the production of the picture.

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