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硼替佐米治疗移植肾中复发性增殖性肾小球肾炎伴单克隆 IgG 沉积:病例报告及文献复习。

Treatment with bortezomib for recurrent proliferative glomerulonephritis with monoclonal IgG deposits in kidney allograft. Case report and review of the literature.

机构信息

Department of Urology, Tokyo Women's Medical University, 8-1 Kawatacho, Shinjuku-ku, Tokyo, 162-8666, Japan.

Department of Nephrology, Tokyo Women's Medical University, Tokyo, Japan.

出版信息

J Nephrol. 2022 May;35(4):1289-1293. doi: 10.1007/s40620-022-01332-x. Epub 2022 May 6.

Abstract

Proliferative glomerulonephritis with monoclonal immunoglobulin IgG deposits (PGNMID) is an already described form of renal involvement by monoclonal gammopathy. PGNMID is known to recur in kidney allografts. Bortezomib has shown clinical success in the treatment of multiple myeloma. However, its effect for recurrent PGNMID in kidney allografts has rarely been reported. We present the case of a 61-year-old woman who developed recurrent PGNMID 3 weeks after kidney transplantation. This patient was initially treated with steroid pulses (500 mg/day for 2 days) and two cycles of rituximab therapy (200 mg/body). However, disease progression was observed with mesangial matrix expansion and subendothelial deposits by light microscopy and stronger staining for IgG3 and kappa in the mesangial area by Immunofluorescence (IF) microscopy. Thus, we started treatment with bortezomib therapy (1.3 mg/m2, once weekly, on days 1, 8, 15, and 22 in a 5-week cycle, for a total of six cycles). Bortezomib therapy reduced massive proteinuria, although monoclonal immune deposits on IF and the serum creatinine level did not change during the treatment period. Seven months after completion of the first bortezomib course, we decided to prescribe a second course of bortezomib with the same regimen. Each course resulted in a > 50% reduction of proteinuria. Bortezomib may delay the progress of PGNMID in kidney allograft patients.

摘要

伴有单克隆免疫球蛋白 IgG 沉积的增生性肾小球肾炎(PGNMID)是单克隆丙种球蛋白病引起的肾脏受累的一种已描述的形式。已知 PGNMID 会在肾移植移植物中复发。硼替佐米在多发性骨髓瘤的治疗中显示出临床疗效。然而,其在肾移植移植物复发性 PGNMID 中的作用很少有报道。我们报告了 1 例 61 岁女性,她在肾移植后 3 周发生复发性 PGNMID。该患者最初接受了类固醇脉冲治疗(500mg/天,2 天)和利妥昔单抗治疗两个周期(200mg/体)。然而,通过光镜观察到系膜基质扩张和内皮下沉积物,免疫荧光(IF)显微镜下系膜区 IgG3 和 kappa 染色增强,观察到疾病进展。因此,我们开始使用硼替佐米治疗(1.3mg/m2,每周 1 次,在 5 周周期的第 1、8、15 和 22 天,共 6 个周期)。硼替佐米治疗减少了大量蛋白尿,尽管 IF 上的单克隆免疫沉积物和血清肌酐水平在治疗期间没有变化。第一个硼替佐米疗程完成后 7 个月,我们决定用相同的方案开第二个硼替佐米疗程。每个疗程均使蛋白尿减少超过 50%。硼替佐米可能会延迟肾移植移植物患者 PGNMID 的进展。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d598/9107408/20fb5720510f/40620_2022_1332_Fig1_HTML.jpg

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