Abstract
B cells play a pivotal role in autoimmunity not only by producing pathogenic autoantibodies but also by modulating immune responses via the production of cytokines and chemokines. The B cell-activating factor/a proliferation-inducing ligand (BAFF/APRIL) system promotes B cell survival and differentiation and thus plays a prominent role in the pathogenesis of autoimmune diseases. Currently, BAFF and APRIL inhibitors are in clinical trials for systemic lupus erythematosus with significant efficacy. However, several studies have demonstrated the efficacy of the BAFF/APRIL blockade which showed considerable variability in the response to B cell-targeted therapy. This may indicate substantial heterogeneity in the pathogenesis of autoimmune diseases. Therefore, objective markers that can predict the effect of BAFF/APRIL-blocking agents could be valuable to the precision medicine linked clinically and to cost-effective therapy.
Highlights
Systemic autoimmune diseases are pathologically characterized by immune complexes consisting of antigens, the activation of dendritic cells and autoreactive T cells, and the overproduction of autoantibodies secreted from activated B cells, which cause severe inflammation in various organs [1]
We reported previously that the proportions of CD19+IgD−CD27+ class-switched memory B cells and CD19+IgD−CD27− effector memory B cells tended to be higher in the peripheral blood of refractory systemic lupus erythematosus (SLE) patients than in that of the control [16,17,18]
B cell-activating factor (BAFF) has been found to be elevated in the serum of associated vasculitis (AAV) patients [45, 46]. These results suggest a potential therapeutic strategy for patients with systemic autoimmune diseases by BAFF and/ or a proliferation-inducing ligand (APRIL) blockade
Summary
Systemic autoimmune diseases are pathologically characterized by immune complexes consisting of antigens, the activation of dendritic cells and autoreactive T cells, and the overproduction of autoantibodies secreted from activated B cells, which cause severe inflammation in various organs [1]. High proportions of circulating Tfh cells, which are characterized as CD4+CXCR5+ICOShighPD-1high, have been described in SLE patients, and their level in the peripheral blood correlates with titers of autoantibodies and with disease severity [31, 32]. Taken together, these findings highlight the notion that activated T cells, in addition to activated B cells, may be potentially involved in the pathogenesis of autoimmunity and that the interaction between activated B and Tfh cells may play an important role in autoantibody-driven autoimmune diseases. The serum level of BAFF and APRIL is both elevated in patients with SLE and positively correlates with disease activity and serological markers such as anti-
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