Expanded CD4+CXCR5-PD-1+peripheral T helper like cells and clinical significance in IgG4-related disease

To identify the phenotypic features and clinical significance of peripheral T helper (Tph) like cells in IgG4-RD, 54 untreated IgG4-RD patients and 57 healthy controls (HCs) were enrolled. Flow cytometry analysis, ELISA and correlation analysis were performed. Results indicated that percentages of CD4 + CXCR5-PD-1+ Tph like cells in the peripheral blood of IgG4-RD patients were significantly higher than those of HCs (2.27% +/- 1.99% vs 1.12% +/- 0.98%, P < 0.001). Expression of CD38, CD25, and TIGIT was higher, whereas that of CCR7, CD127 was lower in the Tph like cells from the IgG4-RD patients than in those from the HCs. The IgG4-RD patients with affected internal organs had higher circulating Tph like cell levels than those without (2.69% +/- 1.99% vs 1.23% +/- 0.93%, respectively, P = 0.003). In addition, Tph like cells correlated with serum IgG and IgG4 and peripheral plasmablast levels which could be a promising biomarker for disease activity monitoring.