Expansion of extrafollicular B and T cell subsets in childhood-onset systemic lupus erythematosus.

ABSTRACT:

Introduction

Most childhood-onset SLE patients (cSLE) develop lupus nephritis (cLN), but only a small proportion achieve complete response to current therapies. The prognosis of children with LN and end-stage renal disease is particularly dire. Mortality rates within the first five years of renal replacement therapy may reach 22%. Thus, there is urgent need to decipher and target immune mechanisms that drive cLN. Despite the clear role of autoantibody production in SLE, targeted B cell therapies such as rituximab (anti-CD20) and belimumab (anti-BAFF) have shown only modest efficacy in cLN. While many studies have linked dysregulation of germinal center formation to SLE pathogenesis, other work supports a role for extrafollicular B cell activation in generation of pathogenic antibod

SUBMITTER: Baxter RM 

PROVIDER: S-EPMC10641733 | biostudies-literature | 2023

REPOSITORIES: biostudies-literature