Immunological mechanism of IgG4-related disease

Changyan Liu; Panpan Zhang; Wen Zhang

doi:10.1016/j.jtauto.2020.100047

Immunological mechanism of IgG4-related disease

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

Authors

Changyan Liu^{1

2}, Panpan Zhang², Wen Zhang²

Affiliations

¹ Department of Rheumatology, The Second Hospital of Dalian Medical University, Dalian, 116023, China.
² Department of Rheumatology, Peking Union Medical College Hospital, National Clinical Research Center for Dermatologic and Immunologic Diseases, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Beijing, 100730, China.

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.