当前位置: X-MOL 学术J. Am. Soc. Nephrol. › 论文详情
Our official English website, www.x-mol.net, welcomes your feedback! (Note: you will need to create a separate account there.)
Recurrence of Anti-Semaphorin 3B-Mediated Membranous Nephropathy after Kidney Transplantation
Journal of the American Society of Nephrology ( IF 13.6 ) Pub Date : 2022-03-01 , DOI: 10.1681/asn.2021101323
Marc Fila 1 , Hanna Debiec 2 , Hélène Perrochia 3 , Nabila Djouadi 1 , Marie-Christine Verpont 2 , David Buob 2, 4 , Pierre Ronco 2, 5
Affiliation  

Background

Membranous nephropathy (MN) is rare in pediatric patients, although its diagnosis may be underestimated in children who are responsive to corticosteroid therapy prescribed for a suspicion of minimal change disease. It is most often associated with an autoimmune disease, predominantly lupus. We previously reported the occurrence of early-onset MN associated with semaphorin 3B in nine children and two adults.

Methods

Biopsies were performed on native kidney and at 1 and 5 months after transplantation. Semaphorin 3B antigen was detected in immune deposits by immunohistochemistry and confocal microscopy on paraffin-embedded biopsies. Anti-semaphorin antibodies were detected by Western blot and analyzed sequentially.

Results

We report the first case of early recurrence after transplantation in a 7-year-old boy who presented with severe nephrotic syndrome and advanced kidney failure. There was no evidence of hereditary or associated autoimmune disease. Abundant, almost coalescent deposits were seen by electron microscopy and bright granular, subepithelial staining was observed for semaphorin 3B antigen. Western blot analysis of serum revealed anti-semaphorin 3B antibodies. Recurrence of MN occurred 25 days after transplantation and manifested as nephrotic range proteinuria despite conventional immunosuppressive therapy. Kidney biopsies confirmed histologic MN recurrence with colocalization of semaphorin 3B antigen and IgG. The patient was treated with rituximab. Anti-semaphorin 3B antibodies, which were detected at transplantation, were not detected 40 days after rituximab.

Conclusion

This case provides evidence that anti-semaphorin 3B antibodies are pathogenic and should be monitored in patients with MN.



中文翻译:

肾移植后抗信号蛋白 3B 介导的膜性肾病的复发

背景

膜性肾病 (MN) 在儿科患者中很少见,但在因疑似微小病变而对皮质类固醇治疗有反应的儿童中,其诊断可能被低估。它通常与自身免疫性疾病有关,主要是狼疮。我们之前曾报道过 9 名儿童和 2 名成人中与信号蛋白 3B 相关的早发性 MN 的发生。

方法

对天然肾脏以及移植后 1 个月和 5 个月进行活检。通过免疫组织化学和石蜡包埋活组织检查的共聚焦显微镜在免疫沉积物中检测到信号素 3B 抗原。通过蛋白质印迹检测抗信号蛋白抗体并依次进行分析。

结果

我们报告了第一例移植后早期复发的 7 岁男孩,他患有严重的肾病综合征和晚期肾衰竭。没有遗传性或相关自身免疫性疾病的证据。通过电子显微镜观察到大量几乎聚结的沉积物,并且观察到信号蛋白 3B 抗原的明亮颗粒状上皮下染色。血清的蛋白质印迹分析揭示了抗信号素 3B 抗体。MN 复发发生在移植后 25 天,尽管进行了常规免疫抑制治疗,但仍表现为肾病范围蛋白尿。肾脏活检证实组织学 MN 复发,信号蛋白 3B 抗原和 IgG 共定位。患者接受了利妥昔单抗治疗。在移植时检测到的抗信号蛋白 3B 抗体,

结论

该病例提供了抗脑信号蛋白 3B 抗体具有致病性的证据,应对 MN 患者进行监测。

更新日期:2022-03-01
down
wechat
bug