Division of Nephrology, Department of Internal Medicine, Seoul St. Mary's Hospital, College of Medicine, The Catholic University of Korea, Seoul, Korea.
Division of Nephrology, Department of Internal Medicine, Yeungnam University Hospital, The Yeungnam University, Daegu, Korea.
Kidney Res Clin Pract. 2013 Sep;32(3):121-6. doi: 10.1016/j.krcp.2013.07.004. Epub 2013 Aug 30.
Nephrotic syndrome (NS) and proteinuria are uncommon, often unrecognized manifestations of graft-versus-host disease (GVHD) after hematopoietic stem cell transplantation (HSCT). Only a few isolated case reports and case series involving smaller number of patients who developed NS after HSCT have been published.
We reviewed the renal histopathological examination findings and clinical records of 15 patients who developed proteinuria after HSCT at Seoul and Yeouido St. Mary's Hospital (Seoul, Korea). We also measured the anti-PLA2R antibodies (M-type phospholipase A2 receptor) in the serum samples from the seven patients at the time of renal biopsy.
All patients had GVHD. The most common indication for biopsy was proteinuria (>1 g/day), with nine patients having nephrotic range proteinuria. The most common histopathological finding was membranous nephropathy (MN; n = 12). Other findings were membranoproliferative glomerulonephritis, C1q nephropathy, and diabetic nephropathy. Eleven patients were treated with immunosuppressive agents, and three patients were treated only with angiotensin II receptor blocker. The overall response rate, including complete remission (urinary protein level <0.3 g/day) and partial remission (urinary protein level = 0.31-3.4 g/day), was 73%. The mean follow-up period was 26 months, and none of the patients developed end-stage renal disease. All of the seven patients with MN had negative findings for anti-PLA2R antibodies, measured using an enzyme-linked immunosorbent assay kit.
In this study the findings of 15 renal biopsies were analyzed and to our knowledge this is the largest clinicopathological study of GVHD-related biopsy-proven nephropathy. Approximately 80% of the patients were MN and 73% responded either partially or completely to immunosuppressive treatment. Currently, there is an increase in the incidence of GVHD-mediated renal disease, and therefore, renal biopsy is essential for diagnosing the nephropathy and preventing the progression of renal disease.
肾病综合征(NS)和蛋白尿是造血干细胞移植(HSCT)后移植物抗宿主病(GVHD)不常见但常被忽视的表现。仅有少数孤立的病例报告和病例系列涉及少数 HSCT 后发生 NS 的患者。
我们回顾了在韩国首尔和汝矣岛圣玛丽医院接受 HSCT 后发生蛋白尿的 15 例患者的肾组织病理学检查结果和临床记录。我们还在肾活检时测量了 7 例患者血清中的抗 PLA2R 抗体(M 型磷脂酶 A2 受体)。
所有患者均有 GVHD。活检的最常见指征是蛋白尿(>1g/天),9 例患者出现肾病范围蛋白尿。最常见的组织病理学表现是膜性肾病(MN;n=12)。其他表现包括膜增生性肾小球肾炎、C1q 肾病和糖尿病肾病。11 例患者接受免疫抑制剂治疗,3 例患者仅接受血管紧张素 II 受体阻滞剂治疗。完全缓解(尿蛋白水平<0.3g/天)和部分缓解(尿蛋白水平=0.31-3.4g/天)的总反应率为 73%。平均随访时间为 26 个月,无患者进展至终末期肾病。MN 的 7 例患者均使用酶联免疫吸附试验试剂盒检测抗 PLA2R 抗体阴性。
本研究分析了 15 例肾活检的结果,据我们所知,这是最大的关于 GVHD 相关性活检证实的肾病的临床病理研究。约 80%的患者为 MN,73%的患者对免疫抑制治疗有部分或完全反应。目前,GVHD 介导的肾脏疾病发病率增加,因此,肾活检对于诊断肾病和防止肾病进展至关重要。