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Immunoglobulin G4-related disease (IgG4RD) is a novel clinical disease entity characterised by elevated serum IgG4 and tissue infiltration by IgG4-positive plasma cells.1 ,2 Interleukin 4 (IL-4) and IL-10, which were detected with B cells in the salivary gland in this disease,3 direct naive B cells to switch to IgG4 production.4 B cells are therefore considered to be important for the pathogenesis of IgG4RD. However, the phenotype of B cells in patients with IgG4RD remains elusive. In this report we show the phenotypic changes of peripheral blood B cells in a patient with IgG4RD analysed by flow cytometry during treatment with a corticosteroid.
In January 2011 a 53-year-old man presented with symmetrical swelling of the lachrymal glands and a tumour located in the left junction of the renal pelvis and ureter, serum IgG4 >135 mg/dl and IgG4+/IgG+ cells >40% with significant invasion of lymphocytes and plasma cells, typical tissue fibrosis and sclerosis in the salivary gland. He was diagnosed with …