Repository Corticotropin in Treating de novo C3 Glomerulonephritis after Transplantation.

Muhammad Saad Naseer, Ayush Singh, Neeraj Singh
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Abstract

Introduction: De novo C3 glomerulonephritis (C3GN) after transplant is uncommon. Although eculizumab has been used successfully in several cases, the response is heterogeneous, and treatment strategies remain undefined. The use of repository corticotropin in C3GN has not been described in the literature.

Case report: A 48-year-old African American male with kidney transplantation secondary to presumed diabetic nephropathy presented 6 years after transplant with lower extremity edema and nephrotic range proteinuria. His urine protein to creatinine ratio (UPCR) was 8.2 g/g. Renal allograft biopsy confirmed the diagnosis of C3GN. He was treated with eculizumab (Solaris®) 900 mg IV once weekly for 4 weeks and repository corticotropin (H.P. Acthar® gel) 80 units SQ twice weekly for 6 months with a near-complete resolution of proteinuria within 3 months of the treatment. The patient presented again 6 months after completing the therapy with a recurrence of proteinuria, which peaked at 11.6 g/g of UPCR. Repeat kidney allograft biopsy was consistent with C3GN. He was started on repository corticotropin 80 units SQ twice weekly, which resulted in a reduction of proteinuria to >50% within 2 months of therapy. When eculizumab 900 mg IV weekly for 4 weeks was added with repository corticotropin, the proteinuria resolved within 10 weeks of treatment. The patient was maintained on monotherapy of repository corticotropin and has been in complete remission of proteinuria for more than a year until his last follow-up.

Conclusion: This is the first case report describing the role of repository corticotropin as an effective therapy in reducing proteinuria and maintaining patients with C3GN in proteinuria remission.

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储存库促肾上腺皮质激素治疗移植后新生C3肾小球肾炎。
摘要:移植后重新发生C3肾小球肾炎(C3GN)并不常见。尽管eculizumab在一些病例中成功使用,但反应是不均匀的,治疗策略仍然不明确。在C3GN中使用储存库促肾上腺皮质激素尚未在文献中描述。病例报告:一例48岁非裔美国男性肾移植继发于糖尿病肾病,移植后6年出现下肢水肿和肾病范围蛋白尿。尿蛋白/肌酐比值(UPCR) 8.2 g/g。肾移植活检证实了C3GN的诊断。他接受了eculizumab (Solaris®)900 mg IV,每周1次,持续4周,以及储存库促肾上腺皮质激素(hp Acthar®凝胶)80单位SQ,每周2次,持续6个月,治疗后3个月内蛋白尿几乎完全消除。患者在完成治疗6个月后再次出现蛋白尿复发,峰值为11.6 g/g UPCR。重复肾移植活检符合C3GN。患者开始使用储存库促肾上腺皮质激素80单位SQ,每周两次,治疗2个月内蛋白尿减少到>50%。当eculizumab 900 mg IV每周,连续4周加入储存库促肾上腺皮质激素时,蛋白尿在治疗10周内消失。患者持续使用储存库促肾上腺皮质激素单药治疗,到最后一次随访时,蛋白尿已完全缓解一年多。结论:这是第一个描述储存库促肾上腺皮质激素在减少蛋白尿和维持C3GN患者蛋白尿缓解中的有效治疗作用的病例报告。
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