Efficacy and Safety of Telitacicept as an Add-On Therapy for Refractory Immunoglobulin A Nephropathy or Immunoglobulin A Vasculitis Nephropathy in Children
Jiaojiao Liu, Xinli Han, Xiaoyun Jiang, Xia Gao, Guomin Li, Xiaoyan Fang, Jing Chen, Yihui Zhai, Jialu Liu, Yuxin Pei, Jiayi Zhang, Guoqin Zhu, Qian Shen, Hong Xu
Abstract
IgA nephropathy (IgAN) and IgA vasculitis nephritis (IgAVN) represent prevalent forms of glomerulonephritis in pediatric population, with significant risk of progression to renal failure. The pathogenesis of both conditions is explained by the four-hit hypothesis: 1) elevated galactose-deficient IgA1 (Gd-IgA1), 2) anti-Gd-IgA1 IgG autoantibody formation, 3) Gd-IgA1-containing immune complexes development, and 4) glomerular deposition of these complexes leading to renal injury. Gd-IgA1-containing immune complexes are central to disease pathogenesis1,2, with B-cell lymphocyte stimulator (BLyS) and a proliferation-inducing ligand (APRIL) playing crucial roles in Gd-IgA1 generation.