Naseer Muhammad Saad, Singh Ayush, Singh Neeraj
Department of Transplant, John C. McDonald Regional Transplant Center-Willis Knighton Health System, Shreveport, Louisiana, USA.
Glomerular Dis. 2021 Oct 22;2(2):100-105. doi: 10.1159/000520387. eCollection 2022 Apr.
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.
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.
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.
移植后新发C3肾小球肾炎(C3GN)并不常见。尽管依库珠单抗已在数例患者中成功使用,但其反应存在异质性,治疗策略仍不明确。文献中尚未描述长效促肾上腺皮质激素在C3GN中的应用。
一名48岁的非裔美国男性,因推测的糖尿病肾病接受肾移植,移植后6年出现下肢水肿和肾病范围蛋白尿。他的尿蛋白肌酐比(UPCR)为8.2 g/g。同种异体肾活检确诊为C3GN。他接受了依库珠单抗(Solaris®)900 mg静脉注射,每周一次,共4周,以及长效促肾上腺皮质激素(H.P. Acthar®凝胶)80单位皮下注射,每周两次,共6个月,治疗3个月内蛋白尿几乎完全缓解。患者在完成治疗6个月后再次出现蛋白尿复发,UPCR峰值达到11.6 g/g。重复肾活检结果与C3GN一致。他开始接受长效促肾上腺皮质激素80单位皮下注射,每周两次,治疗2个月内蛋白尿减少超过50%。当每周一次静脉注射依库珠单抗900 mg共4周并联合长效促肾上腺皮质激素时,蛋白尿在治疗10周内消失。患者维持长效促肾上腺皮质激素单药治疗,直至最后一次随访时蛋白尿已完全缓解超过一年。
这是首例描述长效促肾上腺皮质激素作为有效治疗方法在降低蛋白尿和维持C3GN患者蛋白尿缓解方面作用的病例报告。