Abstract

Regulatory B cells (Bregs) are critical in maintaining self-tolerance. Their role in dermatomyositis (DM), an autoimmune disease characterized by inappropriate regulation of hyperactivated B and T cells, has not been clearly defined. In the current study, we performed flow cytometry analysis of studied CD19+ CD24highCD38high Breg subpopulations in blood samples from 30 patients with DM, 37 diseased controls and 23 healthy controls. A significant decrease was observed in the frequency of Bregs in DM patients compared to that in diseased controls (p < 0.0001) and in healthy controls (p < 0.0001). And the prevalence of Bregs deficiency (defined as Bregs/B cells < 0.50% in this study) in DM patients went as high as 73.3%. Furthermore, DM patients with positive myositis specific autoantibody often had lower Bregs levels than negative patients (p = 0.036), and lower level of Bregs was also found in DM patients with interstitial lung disease than in DM patients without (p = 0.041). In a follow-up study, seven DM patients were considered to be in remission stage, and their Breg levels were found to have significantly increased after treatment (p = 0.022). Our research revealed that Breg deficiency is an immunopathogenic feature of DM and provided insights into the design of new immunotherapy target for DM clinical interventions.

Highlights

  • To date there is no data about the role of Bregs in the pathogenesis of DM, and the number and significance of Bregs in DM remains unclear

  • Peripheral blood lymphocytes from 30 patients with DM, 37 patients with other autoimmune diseases (15 cases of SLE, 15 cases of RA and 7 cases of pSS), and 23 healthy individuals were phenotypically analyzed by flow cytometry for their expression of CD19, CD24, and CD38 surface markers

  • We observed a significant decrease in the levels of CD19+ CD24highCD38high Bregs in DM patients compared to those in healthy controls (0.713 ± 0.275% vs 4.196 ± 0.347%, p < 0.0001) and to those in diseased controls (0.713 ± 0.275% vs 4.010 ± 0.566%, p < 0.0001)

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Summary

Introduction

To date there is no data about the role of Bregs in the pathogenesis of DM, and the number and significance of Bregs in DM remains unclear. Given the fact that both cellular and humoral immunity have been implicated in the pathogenesis of DM, and considering the key role that Bregs play in immunomodulation, it is reasonable to speculate that Bregs may play a pivotal role in the pathomechanism of DM. Our study sought to investigate the presence and phenotypic characteristics of CD19+ CD24highCD38high B lymphocytes in the peripheral blood of patients with DM, and their implication in the extent of the disease activity

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