Pathogenic Mechanisms Underlying Idiopathic Pulmonary Fibrosis — moss 2022

Idiopathic pulmonary fibrosis (IPF) pathogenesis is mediated by a complicated combination of cell types and signaling pathways. Recurrent alveolar epithelial cell (AEC) injury can result in metabolic dysfunction, senescence, abnormal epithelial cell activation, and dysregulated epithelial repair when it is accompanied by predisposing variables (such as genetic, environmental, epigenetic, immunologic, and gerontologic). Multiple signaling systems used by the dysregulated epithelial cell interact with mesenchymal, immunological, and endothelial cells to cause fibroblast and myofibroblast activation. The epithelial damage concept is supported by recent investigations using single-cell RNA sequencing on IPF lungs. These research have discovered a brand-new subtype of AEC that resembles an abnormal basal cell and may interfere with normal epithelium healing and spread a profibrotic phenotype. In this article, we examine the IPF pathogenesis in the context of cutting-edge bioinformatics methods as a means of identifying disease pathways, cell-specific processes, and cell-cell interactions that spread the profibrotic niche.

Moss BJ, Ryter SW, Rosas IO. Pathogenic Mechanisms Underlying Idiopathic Pulmonary Fibrosis. Annu Rev Pathol. 2022 Jan 24;17:515-546. doi: 10.1146/annurev-pathol-042320-030240. Epub 2021 Nov 23. PMID: 34813355.