Abstract：
The recurrence of primary focal segmental glomerulosclerosis (FSGS) after kidney transplantation is associated with a high graft loss rate with standard treatments based on plasmapheresis with/without rituximab. We present 2 consecutive cases of nongenetic early severe recurrent FSGS refractory to rituximab and anti-interleukin 1 treatment and with a partial response to plasmapheresis. Case 1 was a 22-year-old man who was rescue-treated for recurrence 36 weeks after transplantation with obinutuzumab (1000 mg/1.73 m2, 1 dose) and daratumumab (18 mg/kg each dose, 8 doses), resulting in plasmapheresis discontinuation and a drop of proteinuria from 29 to 2.3 g/d. Proteinuria increased with circulating CD38+ plasma cells and responded to an additional daratumumab dose. Currently, the proteinuria is 1.8 g/d, 14.5 months after discontinuing plasmapheresis and starting obinutuzumab and daratumumab therapy. Case 2 was a 15-year-old girl who was plasmapheresis dependent with 2 g/d proteinuria 82 weeks after transplantation, with a Tesio catheter in the right jugular vein as the only possible vascular access. After treatment with obinutuzumab and daratumumab (1 dose each), she achieved stable complete remission (0.3 g/d proteinuria) with persistent plasmapheresis discontinuation. These cases suggest the potential of combining obinutuzumab with daratumumab for the treatment of recurrent FSGS.
摘要：
肾移植后原发性局灶性节段肾小球硬化（FSGS）的复发与基于有/没有利妥昔单抗的血浆置换的标准治疗的高移植物损失率相关。我们介绍了两例对利妥昔单抗和抗白介素-1治疗无效的非遗传早期严重复发性FSGS，并且对血浆置换有部分反应。病例1：一名22岁的男性在移植后36周用奥比努珠单抗（1000mg/1.73m2，一剂）和达雷妥单抗（每剂18mg/kg，八剂)，导致血浆置换停药和蛋白尿从29降至2.3g/天。蛋白尿随着循环CD38+浆细胞增加，并对额外的达雷妥单抗剂量有反应。目前,蛋白尿为1.8克/天，在停止血浆置换并开始奥比努珠单抗和达拉图单抗治疗后14.5个月。案例2：一名15岁女孩在移植82周后依赖血浆置换，2g/天蛋白尿，在右颈静脉使用Tesio®导管作为唯一可能的血管通路。在用奥比努珠单抗和达拉图单抗治疗后（各一剂），她通过持续的血浆置换停药实现了稳定的完全缓解(0.3g/天的蛋白尿).这些病例表明，奥比努珠单抗与达雷木单抗联合治疗复发性FSGS的潜力。
