The Role of Immune Cells in the Pathogenesis of Idiopathic Pulmonary Fibrosis

Abstract

Idiopathic pulmonary fibrosis (IPF) is a devastating disease of unknown etiology with limited treatment options. The role of the immune system in IPF has received increasing attention. Uncontrolled immune responses drive the onset and progression of IPF. This article provides an overview of the role of innate immune cells (including macrophages, neutrophils, mast cells, eosinophils, dendritic cells, nature killer cells, nature kill cells and γδ T cells) and adaptive immune cells (including Th1 cells, Th2 cells, Th9 cells, Th17 cells, Th22 cells, cytotoxic T cells, B lymphocytes and Treg cells) in IPF. In addition, we review the current status of pharmacological treatments for IPF and new developments in immunotherapy. A deeper comprehension of the immune system’s function in IPF may contribute to the development of targeted immunomodulatory therapies that can alter the course of the disease.

Molecular Mechanisms Responsible for the Therapeutic Potential of Mesenchymal Stem Cell-Derived Exosomes in the Treatment of Lung Fibrosis

“…However, the limited availability of suitable donor lungs and lifelong commitment to post-transplant immunosuppressive treatment limit the feasibility of lung transplantation. Despite immunosuppressant medications, the body's immune system may still recognize the transplanted lung as foreign and mount an allogeneic immune response, leading to organ rejection [14,15]. Immunosuppressive drugs down-regulate the production of cytokines, which regulate the proliferation and activation of immune cells and attenuate immune cell-dependent protection against microbial pathogens [16].…”