Pathogenesis of IgG4-related disease: a critical review

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20 Citations (Scopus)


Immunoglobulin G4 (IgG4)-related disease (IgG4-RD) is a chronic, systemic, inflammatory condition of unknown etiology. Histopathologic examination is the key to diagnosis of IgG4-RD. The histopathologic features of IgG4-RD are lymphoplasmacytic infiltration, storiform fibrosis, and obliterative phlebitis. As for fewer than 15 years, IgG4-RD has been recognized as a unified diagnostic entity. CD4 + T and B cells, which likely cause organ damage and disabling tissue fibrosis, constitute the major inflammatory cell population in patients with IgG4-RD. Affected patients with active, untreated disease have a marked expansion of IgG4-secreting plasmablasts in the blood. Important mechanistic insights regarding the pathogenesis of IgG4-RD have been gradually disclosed in recent years. Exploring the role of interactions between these CD4 + T and B cells in patients with IgG4-RD is a highly promising field of investigation. In this review, we focus on CD4 + T cell subsets and the T-cell clones that are involved in the pathogenesis of IgG4-RD.

Original languageEnglish
Pages (from-to)127-132
Number of pages6
Issue number2
Publication statusPublished - Apr 15 2019

All Science Journal Classification (ASJC) codes

  • General Dentistry


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