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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 …
Competing interests YT has received consulting fees, speaking fees and/or honoraria from Mitsubishi-Tanabe Pharma, Chugai Pharma, Eisai Pharma, Pfizer, Abbott Immunology Pharma, Daiichi-Sankyo, Janssen Pharma, Astra-Zeneca, Takeda Industrial Pharma, Astellas Pharma, Asahi-kasei Pharma and GlaxoSmithKline and has received research grant support from Mitsubishi-Tanabe Pharma, Bristol-Myers Squibb, Takeda Industrial Pharma, MSD, Astellas Pharma, Eisai Pharma, Chugai Pharma, Pfizer and Daiichi-Sankyo. The other authors declare no conflict of interest.
Patient consent Obtained.
Ethics approval Ethics approval was obtained from the committee in University of Occupational and Environmental Health, Japan.
Provenance and peer review Not commissioned; externally peer reviewed.