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Background <p>Approximately 95% of patients with idiopathic nephrotic syndrome (INS) have a favorable prognosis, though 4–5% progress to end-stage renal disease. The recurrence rate of INS following kidney transplantation is approximately 30%. Treating nephrotic syndrome (NS) after kidney transplantation is challenging and often results in graft loss. In the present case, following transplantation, the patient experienced recurrent NS caused by podocytopathy in which elevated anti-nephrin antibody levels were closely associated with the degree of proteinuria. We present this case and discuss it in the context of the existing literature.</p> Case presentation <p>The patient was a 59-year-old male who first developed INS in 1980 at the age of 15 and was diagnosed with minimal change disease (MCD) on the basis of a renal biopsy. He initially achieved complete remission with steroids, but gradually developed steroid resistance and started hemodialysis in 2006. In 2014, he received a living-donor kidney transplant from his younger brother, but in 2017 his NS recurred. A biopsy of the transplanted kidney revealed MCD. The biopsy results were MCD, but clinically, it was FSGS, and low-density lipoprotein cholesterol apheresis (LDL-A) was performed 12 times per course for a total of four courses. However, his proteinuria persisted and he continued to have NS. In December 2023, a blood test revealed elevated levels of anti-nephrin antibody. In January 2024, following steroid pulse therapy with methylprednisolone at 500&#xa0;mg/day for 3&#xa0;days combined with a single rituximab administration at 200&#xa0;mg/body, the anti-nephrin antibodies became undetectable, and the proteinuria resolved. Subsequent super-resolution microscopic examination of a biopsy specimen collected before treatment revealed co-localization of immunoglobulin (Ig)G and nephrin.</p> Conclusions <p>We report a case of recurrent NS caused by podocytopathy following renal transplantation in a patient who tested positive for anti-nephrin antibodies. Biopsy of the transplant kidney revealed co-localization of IgG and nephrin. Combined steroid pulse therapy and treatment with rituximab induced complete remission, with the anti-nephrin antibody titer correlating with the proteinuria volume.</p>

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Nephrotic syndrome caused by recurrent podocytopathy after living donor renal transplantation with elevated anti-nephrin antibody levels: a case report and a review

  • 期刊论文

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  • 发表日期：

    2025年4月5日

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  • Ryoichi Miyazaki,
  • Kyoko Miyagi,
  • Tatsuhito Miyanaga,
  • Yoko Shirai,
  • Kenichiro Miura,
  • Motoshi Hattori,
  • Satoshi Hara,
  • Kiyoaki Ito,
  • Ichiro Mizushima,
  • Yasunori Iwata

摘要

Background

Approximately 95% of patients with idiopathic nephrotic syndrome (INS) have a favorable prognosis, though 4–5% progress to end-stage renal disease. The recurrence rate of INS following kidney transplantation is approximately 30%. Treating nephrotic syndrome (NS) after kidney transplantation is challenging and often results in graft loss. In the present case, following transplantation, the patient experienced recurrent NS caused by podocytopathy in which elevated anti-nephrin antibody levels were closely associated with the degree of proteinuria. We present this case and discuss it in the context of the existing literature.

Case presentation

The patient was a 59-year-old male who first developed INS in 1980 at the age of 15 and was diagnosed with minimal change disease (MCD) on the basis of a renal biopsy. He initially achieved complete remission with steroids, but gradually developed steroid resistance and started hemodialysis in 2006. In 2014, he received a living-donor kidney transplant from his younger brother, but in 2017 his NS recurred. A biopsy of the transplanted kidney revealed MCD. The biopsy results were MCD, but clinically, it was FSGS, and low-density lipoprotein cholesterol apheresis (LDL-A) was performed 12 times per course for a total of four courses. However, his proteinuria persisted and he continued to have NS. In December 2023, a blood test revealed elevated levels of anti-nephrin antibody. In January 2024, following steroid pulse therapy with methylprednisolone at 500 mg/day for 3 days combined with a single rituximab administration at 200 mg/body, the anti-nephrin antibodies became undetectable, and the proteinuria resolved. Subsequent super-resolution microscopic examination of a biopsy specimen collected before treatment revealed co-localization of immunoglobulin (Ig)G and nephrin.

Conclusions

We report a case of recurrent NS caused by podocytopathy following renal transplantation in a patient who tested positive for anti-nephrin antibodies. Biopsy of the transplant kidney revealed co-localization of IgG and nephrin. Combined steroid pulse therapy and treatment with rituximab induced complete remission, with the anti-nephrin antibody titer correlating with the proteinuria volume.