Clinical Features and Mechanistic Insights Regarding IgG4-related Dacryoadenitis and Sialoadenitis: a Review

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. Patients with IgG4-RD, who have active, untreated disease, exhibit marked expansion of IgG4-secreting plasmablasts in the blood. Important mechanistic insights correlated with the pathogenesis of IgG4-RD have been disclosed in recent years through the application of novel molecular biology approaches, including next-generation and single-cell RNA sequencing. Exploration of the interactions between these CD4 T cells and cells of the B lymphocyte lineage is critical to understanding the pathophysiology of IgG4-RD. The establishment of pathogenic T cell clones and the identification of antigens specific to these clones constitute the first steps in determining the pathogenesis of this disease. This review focuses on clinical features and mechanistic insights regarding IgG4-related dacryoadenitis and sialoadenitis, from a perspective suitable for oral and maxillofacial surgeons.