Immunoglobulin G4-related disease (IgG4-RD), recognized only recently as a single diagnostic entity, is a chronic inflammatory condition of unknown etiology. The diagnosis of IgG4-RD relies heavily on histopathological analysis and the correlation of histology findings with clinical, serological, and radiological data. CD4+ T and B cells, including IgG4-expressing plasmablasts, constitute the major inflammatory cell populations in IgG4-RD and are believed to cause organ damage and tissue fibrosis.
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