Pediatric Nephrology Institute, Ruth Children's Hospital, Rambam Health Care Campus, Haifa, Israel.
Technion Faculty of Medicine, Haifa, Israel.
Sci Rep. 2024 Oct 26;14(1):25493. doi: 10.1038/s41598-024-74184-z.
Focal segmental glomerulosclerosis (FSGS) is a major cause of pediatric kidney failure. Most cases of FSGS in children are idiopathic and have a high risk of post-transplantation recurrence and graft loss. Common treatments for recurrent FSGS (rFSGS) post-transplantation include plasmapheresis, immunoadsorption, and/or immunomodulatory therapy. This study retrospectively evaluated the efficacy and safety of early plasmapheresis followed by rituximab for inducing and maintaining remission in rFSGS. Between 2014 and 2023, 8 of 65 pediatric kidney transplant recipients at our center were diagnosed with idiopathic FSGS. rFSGS was diagnosed based on nephrotic range proteinuria with no other cause and managed with plasmapheresis. Rituximab therapy was used for those who did not achieve complete remission with prolonged plasmapheresis or remained plasmapheresis dependent. 6 of 8 (75%) transplant recipients with idiopathic FSGS experienced rFSGS. All patients achieved partial or complete remission with plasmapheresis, with response times ranging from 8 to 379 days (median 13 days). Rituximab therapy was introduced for 5 plasmapheresis-dependent patients, leading to sustained remission and cessation of plasmapheresis in 3 patients, while 2 showed improved proteinuria and reduced plasmapheresis frequency. Adverse effects included rituximab-induced serum sickness in one patient and one mild allergic reaction. One patient experienced graft loss due to humoral rejection, but no grafts were lost to rFSGS, and all other grafts remained functional over an average follow-up of 50 months. Early plasmapheresis followed by rituximab therapy effectively induces remission in most post-transplantation rFSGS cases, is well tolerated, and prevents graft loss. Larger studies are needed to confirm these findings.
局灶节段性肾小球硬化症（FSGS）是小儿肾衰竭的主要原因。大多数儿童 FSGS 为特发性，具有移植后复发和移植物丢失的高风险。移植后 FSGS 复发（rFSGS）的常见治疗方法包括血浆置换、免疫吸附和/或免疫调节治疗。本研究回顾性评估了早期血浆置换后使用利妥昔单抗诱导和维持 rFSGS 缓解的疗效和安全性。在 2014 年至 2023 年间，我们中心 65 例小儿肾移植受者中，有 8 例被诊断为特发性 FSGS。rFSGS 的诊断依据是肾病范围内蛋白尿且无其他病因，并采用血浆置换治疗。对于那些经过长时间血浆置换仍未完全缓解或仍依赖血浆置换的患者，使用利妥昔单抗治疗。8 例特发性 FSGS 中有 6 例（75%）发生 rFSGS。所有患者均通过血浆置换获得部分或完全缓解，反应时间为 8 至 379 天（中位数 13 天）。5 例依赖血浆置换的患者采用利妥昔单抗治疗，其中 3 例持续缓解并停止血浆置换，2 例蛋白尿改善且血浆置换频率降低。不良反应包括 1 例患者出现利妥昔单抗诱导的血清病，1 例患者出现轻度过敏反应。1 例患者因体液排斥反应导致移植物丢失，但无移植物因 rFSGS 丢失，所有其他移植物在平均 50 个月的随访中均保持功能。早期血浆置换后使用利妥昔单抗治疗可有效诱导大多数移植后 rFSGS 病例缓解，耐受性良好，可预防移植物丢失。需要更大规模的研究来证实这些发现。
