Allergic Aspects of IgG4-Related Disease: Implications for Pathogenesis and Therapy

Front Immunol. 2021 Jul 7:12:693192. doi: 10.3389/fimmu.2021.693192. eCollection 2021.

Abstract

IgG4-related disease (IgG4-RD) is a rare systemic fibroinflammatory disease frequently associated with allergy. The pathogenesis of IgG4-RD is poorly understood, and effective therapies are limited. However, IgG4-RD appears to involve some of the same pathogenic mechanisms observed in allergic disease, such as T helper 2 (Th2) and regulatory T cell (Treg) activation, IgG4 and IgE hypersecretion, and blood/tissue eosinophilia. In addition, IgG4-RD tissue fibrosis appears to involve activation of basophils and mast cells and their release of alarmins and cytokines. In this article, we review allergy-like features of IgG4-RD and highlight targeted therapies for allergy that have potential in treating patients with IgG4-RD.

Keywords: IgG4-related disease; alarmins; allergy; basophils; eosinophils; mast cells; type 2 immmune response.

Publication types

  • Research Support, N.I.H., Extramural
  • Research Support, N.I.H., Intramural
  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Alarmins / metabolism
  • Animals
  • Cytokines / metabolism
  • Fibrosis
  • Humans
  • Hypersensitivity / immunology*
  • Hypersensitivity / metabolism
  • Hypersensitivity / physiopathology
  • Immune System / immunology*
  • Immune System / metabolism
  • Immune System / physiopathology
  • Immunoglobulin E / metabolism
  • Immunoglobulin G / immunology*
  • Immunoglobulin G / metabolism
  • Immunoglobulin G4-Related Disease / immunology*
  • Immunoglobulin G4-Related Disease / metabolism
  • Immunoglobulin G4-Related Disease / physiopathology
  • Signal Transduction

Substances

  • Alarmins
  • Cytokines
  • Immunoglobulin G
  • Immunoglobulin E