Immunopathogenesis and therapeutic potential of macrophage influx in diffuse parenchymal lung diseases

The diffuse parenchymal lung diseases (DPLD)/interstitial lung diseases (ILD) are progressive lung disorders with usually unclear etiology, poor long-term survival and no effective treatment. Their pathogenesis is characterized by alveolar epithelial cell injury, inflammation, epithelial-mesenchymal transition, and parenchymal fibrosis. Macrophages play diverse roles in their development, both in the acute phase and in tissue repair.

In this review, we summarize the current state of knowledge regarding the role of macrophages and their phenotypes in the immunopathogenesis of DPLDs; CVD-ILD, UIP, NSIP, DIP, RB-ILD, AIP, HP, Sarcoidosis, etc. Our goal is to update the understanding of the immune mechanisms underlying the initiation and progression of fibrosis in DPLDs. This will help in identification of biomarkers and in developing novel therapeutic strategies for DPLDs. A thorough literature search of the published studies in PubMed (from 1975 to 2020) was done.

The macrophage associated inflammatory markers needs to be explored for their potential as biomarkers of disease activity and progression. Pharmacological targeting of macrophage activation may reduce the risk of macrophage activation syndrome (MAS) and help improving the survival and prognosis of these patients.