Orchestration of Immune Cells Contributes to Fibrosis in IgG4-Related Disease

This review summarizes recent progress in understanding the pathogenesis of IgG4-related disease (IgG4-RD), with a focus on fibrosis. Several studies reported that CD4+ T cells cytotoxic activity promoted by secretion granzyme and perforin, (CD4+CTLs), disease-specific activated B cells, infiltrated inflamed tissues cooperated to induce tissue fibrosis autoimmune fibrotic diseases such as IgG4-RD, systemic sclerosis, fibrosing mediastinitis. An accumulation undergoing apoptotic cell death induced CD4+CTLs CD8+CTLs followed macrophage-mediated clearing finally remodeling driven cytokines released CD4+CTLs, M2 macrophages may contribute activation fibroblasts collagen production. In this process likely involves apoptosis non-immune, non-endothelial mesenchymal origin subsequent remodeling. summary, infiltrate affected where they cooperate CD8+CTLs, macrophages, secreting cytokines. These immune also drive pro-fibrotic molecules IgG4-RD.