Bernard Josselin, Bruel Alexandra, Allain-Launay Emma, Dantal Jacques, Roussey Gwenaelle
Pediatric Department, University Hospital of Nantes, Nantes, France.
Nephrology and Immunology Department, University Hospital of Nantes, Nantes, France.
Pediatr Transplant. 2018 Jun;22(4):e13175. doi: 10.1111/petr.13175. Epub 2018 Mar 23.
Treatment of SRNS is a challenge. Antiproliferative agents and depleting antibodies have been reported to be effective. However, these agents are not always successful, and use of ofatumumab could provide a different treatment option. Our patient was diagnosed with a SRNS at 5 years of age. She developed ESRD, with FSGS. This was cause for a first renal transplantation. The NS relapsed, leading to loss of the graft, and a second renal transplantation was performed. Due to the recurrence of the NS, IAds were initiated and led to a complete remission. Our patient remained dependent on IAds, however, despite treatments with calcineurin inhibitors, corticosteroids, rituximab, and abatacept. Ofatumumab was introduced and led to a remission, thus allowing cessation of the IAd treatment. Another infusion of ofatumumab was administered 8 months after the last one, due to the recurrence of the NS and a renewed increase in B cells. Although it did not result in a complete remission, the proteinuria was stabilized in the absence of IAds. Ofatumumab may be an alternative treatment for post-transplantation rituximab-resistant SRNS, although this needs to be confirmed by further studies.
治疗类固醇抵抗型肾病综合征(SRNS)是一项挑战。据报道,抗增殖药物和消耗性抗体有效。然而,这些药物并非总能成功,而使用奥法妥木单抗可能提供一种不同的治疗选择。我们的患者5岁时被诊断为SRNS。她发展为终末期肾病,伴有局灶节段性肾小球硬化(FSGS)。这是首次进行肾移植的原因。肾病综合征复发,导致移植肾丧失,随后进行了第二次肾移植。由于肾病综合征复发,开始使用免疫吸附(IAds)并导致完全缓解。然而,尽管使用了钙调神经磷酸酶抑制剂、皮质类固醇、利妥昔单抗和阿巴西普进行治疗,我们的患者仍依赖IAds。引入奥法妥木单抗后导致缓解,从而可以停止IAds治疗。由于肾病综合征复发且B细胞再次增多,在最后一次输注奥法妥木单抗8个月后又进行了一次输注。尽管未导致完全缓解,但在未使用IAds的情况下蛋白尿得以稳定。奥法妥木单抗可能是移植后利妥昔单抗抵抗型SRNS的一种替代治疗方法,不过这需要进一步研究予以证实。