Abstract
Immunoglobulin G4-related disease (IgG4-RD) is a systemic disorder characterized by tissue fibrosis and intense lymphoplasmacytic infiltration, causing progressive organ dysfunction. Activation-induced cytidine deaminase (AID), a deaminase normally expressed in activated B-cells in germinal centers, edits ribonucleotides to induce somatic hypermutation and class switching of immunoglobulin. While AID expression is strictly controlled under physiological conditions, chronic inflammation has been noted to induce its upregulation to propel oncogenesis. We examined AID expression in IgG4-related ophthalmic disease (IgG4-ROD; n = 16), marginal zone lymphoma with IgG4-positive cells (IgG4+ MZL; n = 11), and marginal zone lymphoma without IgG4-positive cells (IgG4- MZL; n = 12) of ocular adnexa using immunohistochemical staining. Immunohistochemistry revealed significantly higher AID-intensity index in IgG4-ROD and IgG4+ MZL than IgG4- MZL (p < 0.001 and = 0.001, respectively). The present results suggest that IgG4-RD has several specific causes of AID up-regulation in addition to inflammation, and AID may be a driver of oncogenesis in IgG4-ROD to IgG4+ MZL.
Highlights
It has been 20 years since Immunoglobulin (Ig) G4-related disease (IgG4-RD) was first described in 2001 [1]
Immunoglobulin G4-related disease (IgG4-RD) remains unclear; previous research noted that upregulation of cytokines secreted from T-helper 2 (Th2) and regulatory T (Treg) cell, including interleukin (IL) 4, IL-5, IL-10, IL-13, and transforming growth factor-beta 1 (TGF-β1) has been considered to play the crucial role in the onset of the disorder, causing the proliferation of IgG4- plasma cells [5]
We aimed to clarify the extent of Activation-induced cytidine deaminase (AID) expression in ocular adnexal IgG4+ marginal zone lymphoma (MZL) and IgG4-ROD compared with MZL without IgG4-positive cells of ocular adnexa (IgG4- MZL) to see if AID plays a role in the pathogenesis of IgG4+
Summary
It has been 20 years since Immunoglobulin (Ig) G4-related disease (IgG4-RD) was first described in 2001 [1]. It is a systemic disorder characterized by the formation of fibrotic lesions in various organs, including the pancreas, aorta, bile ducts, kidneys, and ocular adnexa [2,3]. Previous studies have argued that patients with IgG4-RD might have an increased risk of malignancies including colorectal, lung, and pancreatic cancer as well as malignant lymphoma than the general population [6]. Some case reports have described the emergence of malignant lymphoma among patients with IgG4-RD [7,8,9,10]
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