Journal
ODONTOLOGY
Volume 107, Issue 2, Pages 127-132Publisher
SPRINGER
DOI: 10.1007/s10266-018-0377-y
Keywords
IgG4-related disease; T follicular helper cell; CD4(+) cytotoxic T lymphocytes; B cell; Plasmablast
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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.
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