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. 2020 Mar 19:3:100047.
doi: 10.1016/j.jtauto.2020.100047. eCollection 2020.

Immunological mechanism of IgG4-related disease

Changyan Liu  1   2 , Panpan Zhang  2 , Wen Zhang  2
Affiliations

Immunological mechanism of IgG4-related disease

Changyan Liu et al. J Transl Autoimmun. .

Abstract

IgG4-related disease (IgG4-RD) is an immune-mediated inflammatory disorder in multiple organs, characterized by abundant infiltration of IgG4-positive plasmacytes and fibrosis in the involved organs. The precise pathogenic mechanism of IgG4-RD still remains unclear. Aberrant innate and adaptive immunity are considered as the main pathogenesis of IgG4-RD. Recent studies have shown that abnormal adaptive immune responses mediated by T helper type 2 ​cells, regulatory T lymphocytes, CD4+ cytotoxic T lymphocytes, T follicular helper cells, T follicular regulatory cells, PD-1hiCXCR5-peripheral T helper cells and B cell subsets are involved in IgG4-RD. In addition to adaptive immune responses, innate immune responses play pathogenic roles in IgG4-RD. Macrophages, mast cells, basophils, complement, and plasmacytoid dendritic cells are activated to produce various kinds of cytokines in IgG4-RD. This review aims to summarize the most recent knowledge in the pathogenesis of IgG4-RD.

Keywords: Adaptive immune; IgG4-related disease; Innate immune; Pathogenesis.

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Conflict of interest statement

The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper.

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