Solomon Sonia, Zolotnitskaya Anna, Del Rio Marcela
Children's Hospital at Montefiore, Bronx, New York.
Pediatr Transplant. 2019 Jun;23(4):e13413. doi: 10.1111/petr.13413. Epub 2019 Apr 11.
FSGS is a potentially devastating form of nephrotic syndrome. Treatment of SRNS can be difficult, especially post-transplantation. The current therapy of post-transplant SRNS includes plasmapheresis, ACE-I, CNI, and monoclonal antibodies (rituximab). Patients who are refractory to these interventions have limited therapeutic alternatives. We present a case of a patient with SRNS secondary to FSGS. He did not respond to immunosuppressive medications prior to transplant, progressed to ESRD, and was started on chronic hemodialysis. He received a DDKT which was complicated by post-transplant FSGS recurrence. A course of plasmapheresis, rituximab, and CNI were administered with some response. Ofatumumab was then given to the patient. As a result, the patient achieved partial remission. Ofatumumab may be a safe and effective option for post-transplant recurrence of FSGS.
局灶节段性肾小球硬化(FSGS)是一种可能具有毁灭性的肾病综合征形式。治疗类固醇抵抗性肾病综合征(SRNS)可能很困难,尤其是在移植后。目前移植后SRNS的治疗方法包括血浆置换、血管紧张素转换酶抑制剂(ACE-I)、钙调神经磷酸酶抑制剂(CNI)和单克隆抗体(利妥昔单抗)。对这些干预措施难治的患者治疗选择有限。我们报告一例继发于FSGS的SRNS患者。他在移植前对免疫抑制药物无反应,进展为终末期肾病(ESRD),并开始进行慢性血液透析。他接受了死亡供者肾移植(DDKT),但并发移植后FSGS复发。给予一个疗程的血浆置换、利妥昔单抗和CNI后有一定反应。然后给该患者使用奥法木单抗。结果,患者实现了部分缓解。奥法木单抗可能是移植后FSGS复发的一种安全有效的选择。