IgG4-related disease and its pathogenesis-cross-talk between innate and acquired immunity

Int Immunol. 2014 Nov;26(11):585-95. doi: 10.1093/intimm/dxu074. Epub 2014 Jul 14.


IgG4-related disease (IgG4-RD) is a novel clinical entity proposed in Japan in the 21th century and is attracting strong attention over the world. The characteristic manifestations of IgG4-RD are increased serum IgG4 concentration and tumefaction by IgG4(+) plasma cells. Although the clinical manifestations in various organs have been established, the pathogenesis of IgG4-RD is still unknown. Recently, many reports of aberrant acquired immunity such as Th2-diminated immune responses have been published. However, many questions still remain, including questions about the pathogenesis of IgG4-RD and the roles of IgG4. In this review, we discuss the pathogenesis of IgG4-RD by focusing on the cross-talk between innate and acquired immunity.

Keywords: IgG4; Th2; regulatory T cell; toll-like receptor.

Publication types

  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Adaptive Immunity*
  • B-Lymphocytes / immunology
  • B-Lymphocytes / metabolism
  • Cell Communication
  • Fibrosis
  • Humans
  • Immune System Diseases / diagnosis
  • Immune System Diseases / etiology*
  • Immunity, Innate*
  • Immunoglobulin Class Switching
  • Immunoglobulin G / immunology*


  • Immunoglobulin G