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To investigate the role of Programmed cell death protein 1 (PD-1) and its two ligands PD-L1 and PD-L2 in the pathogenesis of IgG4-RD. Patients with IgG4-RD (n = 43) as well as healthy controls (n = 34) were recruited. Expression levels of PD-1, PD-L1 and PD-L2 in plasma, submandibular gland and T cell subsets were determined by ELISA, immunohistochemistry and flow cytometry. Naive T cells were stimulated with or without PD-L1/PD-L2 or anti-PD-L1/anti-PD-L2 for 7 days and the proportion of CD4+CD25+Treg cells was detected by flow cytomet