Curr Opin Immunol. 2025 May 20;95:102564. doi: 10.1016/j.coi.2025.102564. Online ahead of print.
ABSTRACT
IgG4-related disease (IgG4-RD), recognized as a novel clinical entity, is a rare, chronic, immune-mediated systemic fibroinflammatory disorder of unknown origin with either synchronous or metachronous multi-organ involvement. Although the pathogenic mechanism remains unclear, possible multipathogenic factors such as genetic backgrounds, disease-specific or related antigens, and abnormal innate or adaptive immunity may be involved. Many immunocytes, including neutrophil extracellular trap, M2 macrophage, plasmablast, B cells, and T-cells (Th2-CD4+T, follicular helper T cells, and CD4+SLAMF7+cytotoxic T cells) play important roles in the pathogenesis. Conventional therapies with glucocorticoid or rituximab in combination with/without immunomodulators are recommended in all symptomatic patients with active IgG4-RD. Because of a few of randomized clinical trials, the comprehensive management for IgG4-RD has not been established yet. Targeted treatment approaches against the plasmablast to B cell lineage and the CD4+SLAMF7+cytotoxic T cell seem to be promising for the future-directed treatment.
PMID:40398200 | DOI:10.1016/j.coi.2025.102564