Xu Hongyao, Huang Yao, Dong Ling, Yu Hua, Lin Bo
Department of Dermatology, Zhejiang Provincial People's Hospital Bijie Hospital, Bijie, Guizhou, China.
The Clinical Medical College, Zunyi Medical University, Zunyi, Guizhou, China.
Front Med (Lausanne). 2024 Jul 24;11:1360979. doi: 10.3389/fmed.2024.1360979. eCollection 2024.
Proliferative glomerulonephritis with monoclonal immunoglobulin deposits (PGNMID) is a rare disease without standardized treatment modalities. Daratumumab is a human IgG monoclonal anti-CD38 antibody that has been demonstrated to be highly effective and safe in the treatment of PGNMID. This article reports a 66-year-old female who suffered from edema in both lower limbs and face for 6 years with mild proteinuria and hypoproteinemia. Renal biopsy displayed eight glomeruli, among which two presented with glomerulosclerosis, and the remaining six exhibited moderate diffuse hyperplasia of glomerular mesangial cells and stroma with endothelial cell proliferation. Immunofluorescence microscopy revealed lumpy and diffuse deposits of C3, C1q, IgG, and κ light chain in the glomerular mesangium, with strongly positive staining for IgG3 and varying degrees of weak to negative staining for IgG1, IgG2, IgG4, and λ light chain. Additionally, ultrastructural analysis unveiled that the glomerular basement membrane was segmentally thickened, accompanied by diffuse pedicle fusion, segmental tethered insertion, subendothelial deposits, and electron-dense material in tethered areas. The patient received a total dose of 800 mg of daratumumab (400 mg daily for two consecutive days), as well as daily prednisone (25 mg) and valsartan (80 mg), for treatment and achieved complete remission after three-month follow-up. This case represents an early attempt to treat PGNMID with low-dose daratumumab but requires long-term follow-up.
伴单克隆免疫球蛋白沉积的增殖性肾小球肾炎（PGNMID）是一种罕见疾病，尚无标准化治疗方案。达雷妥尤单抗是一种人IgG单克隆抗CD38抗体，已被证明在治疗PGNMID方面高效且安全。本文报告了一名66岁女性，双下肢及面部水肿6年，伴有轻度蛋白尿和低蛋白血症。肾活检显示8个肾小球，其中2个呈现肾小球硬化，其余6个表现为肾小球系膜细胞和基质中度弥漫性增生伴内皮细胞增殖。免疫荧光显微镜检查显示肾小球系膜区有C3、C1q、IgG和κ轻链的团块样和弥漫性沉积，IgG3染色强阳性，IgG１、IgG2、IgG4和λ轻链呈不同程度的弱阳性至阴性染色。此外，超微结构分析显示肾小球基底膜节段性增厚，伴有弥漫性足突融合、节段性栓系插入、内皮下沉积物以及栓系区域的电子致密物质。该患者接受了总量800mg的达雷妥尤单抗（连续两天每日400mg）以及每日泼尼松（25mg）和缬沙坦（80mg）治疗，随访3个月后实现完全缓解。该病例是使用低剂量达雷妥尤单抗治疗PGNMID的早期尝试，但需要长期随访。
