Jin Yanyan, Zhu Jianhong, Sheng Aiqin, Lin Qian, Zhao Manli, He Xue, Mao Jianhua, Fu Haidong
Department of Nephrology, the Children'S Hospital, Zhejiang University School of Medicine, National Clinical Research Center for Child Health, #3333 Binsheng Road, Hangzhou, 310052, Zhejiang Province, China.
Department of Nephrology, Quanzhou Maternity and Children'S Hospital, Quanzhou, China.
Pediatr Nephrol. 2025 Apr 11. doi: 10.1007/s00467-025-06769-3.
Persistent proteinuria is an independent risk factor for poor prognosis in immunoglobulin A vasculitis nephritis (IgAVN). Recent studies have shown that telitacicept effectively reduces proteinuria levels in patients with IgA nephropathy (IgAN). The treatment has been associated with a significant decrease in galactose-deficient IgA1 (Gd-IgA1) levels. This study aims to elucidate the effectiveness and safety of telitacicept in treating refractory childhood IgAVN.
This is a single-center, retrospective observational study of seven children with IgAVN who had previously received glucocorticoids and at least one immunosuppressive therapy but still exhibited proteinuria. Telitacicept was administered subcutaneously once a week. Dosage was 80 mg for individuals weighing < 40 kg and 160 mg for those weighing > 40 kg.
The study encompassed seven children, comprising two boys and five girls, with a median age of 15 years. Among the participants, six children (85.7%) exhibited a reduction in proteinuria, ranging from - 23.6 to - 97.5% compared to baseline levels. Conversely, one child (14.3%) did not show any decrease in proteinuria. Throughout the follow-up period, two children (28.6%) achieved complete remission, characterized by proteinuria level below 0.2 g/day/1.73 m and negative dipstick test for blood. Additionally, all participants demonstrated a significant decline in urinary red blood cell counts. In terms of medication administration, all patients discontinued the use of steroids and immunosuppressants. Furthermore, there was a reduction in the incidence of hospitalizations. Importantly, no serious adverse reactions were reported.
Telitacicept has demonstrated both efficacy and safety in treating children with refractory IgAVN.
持续性蛋白尿是免疫球蛋白A血管炎肾病（IgAVN）预后不良的独立危险因素。近期研究表明，泰吉华单抗可有效降低IgA肾病（IgAN）患者的蛋白尿水平。该治疗与半乳糖缺乏型IgA1（Gd-IgA1）水平显著降低有关。本研究旨在阐明泰吉华单抗治疗难治性儿童IgAVN的有效性和安全性。
这是一项单中心回顾性观察研究，研究对象为7例曾接受糖皮质激素及至少一种免疫抑制治疗但仍有蛋白尿的IgAVN儿童。泰吉华单抗每周皮下注射一次。体重<40 kg者剂量为80 mg，体重>40 kg者剂量为160 mg。
该研究纳入7名儿童，其中2名男孩和5名女孩，中位年龄为15岁。参与者中，6名儿童（85.7%）蛋白尿减少，与基线水平相比减少了-23.6%至-97.5%。相反，1名儿童（14.3%）蛋白尿未减少。在整个随访期间，2名儿童（28.6%）实现完全缓解，其特征为蛋白尿水平低于0.2 g/天/1.73 m²且尿潜血试纸检测为阴性。此外，所有参与者的尿红细胞计数均显著下降。在用药方面，所有患者均停用了类固醇和免疫抑制剂。此外，住院率有所降低。重要的是，未报告严重不良反应。
泰吉华单抗在治疗难治性儿童IgAVN方面已证明具有有效性和安全性。
