Mao Youying, Zhang Chenxing, Xu Jing, Liu Chong, Zheng Song Guo, Yin Lei
Department of Nephrology, Shanghai Children's Medical Center, Shanghai Jiaotong University School of Medicine, Shanghai, China.
Department of Pediatrics, Songjiang Hospital Affiliated to the Shanghai Jiaotong University School of Medicine, Shanghai, China.
Pediatr Nephrol. 2025 Jun;40(6):1903-1905. doi: 10.1007/s00467-024-06620-1. Epub 2025 Jan 8.
IgA nephropathy (IgAN) is one of the most common primary glomerular diseases worldwide and is the most common cause of chronic kidney disease (CKD) in China. It lacks recommended treatment in refractory IgAN after conventional therapy. Belimumab, a recombinant human IgG-1λ monoclonal antibody that inhibits the B lymphocyte stimulator, is recommended to treat active lupus nephritis. This report presents a case of a child with severe and refractory IgAN who showed kidney response after belimumab addition combined with conventional therapy. The therapy result was accompanied by decreased plasma cells in peripheral blood. It is the first case showing the benefits of adding belimumab to other immunosuppressive regimens.
IgA肾病（IgAN）是全球最常见的原发性肾小球疾病之一，也是中国慢性肾脏病（CKD）的最常见病因。在传统治疗后，难治性IgAN缺乏推荐的治疗方法。贝利尤单抗是一种重组人IgG-1λ单克隆抗体，可抑制B淋巴细胞刺激因子，被推荐用于治疗活动性狼疮性肾炎。本报告介绍了一例重症难治性IgAN患儿，在联合传统治疗加用贝利尤单抗后肾脏出现反应。治疗结果伴有外周血浆细胞减少。这是首例显示在其他免疫抑制方案中加用贝利尤单抗有益的病例。
