From the Department of Pediatric Nephrology, Baskent University Faculty of Medicine, Ankara, Turkey.
Exp Clin Transplant. 2022 May;20(Suppl 3):49-52. doi: 10.6002/ect.PediatricSymp2022.O15.
Late antibody-mediated rejection triggered by donor-specific antibodies is a leading cause of kidney allograft failure. Effective treatment options for late antibody-mediated rejection are limited in renal transplant recipients. Here, we report 2 pediatric cases of severe late antibody-mediated rejection resistant to conventional immunosuppressive therapy who were successfully treated with eculizumab. Two patients who fulfilled the late antibody-mediated rejection diagnostic criteria (positive donor-specific antibodies, elevated mean fluorescence index, acute and/or chronic morphological lesions in the microvasculature, and abnormal kidney function test) were included in this study. Both patients were previously unsensitized with negative panel-reactive antibody. Case 1 was a 12-year-old male patient with kidney failure secondary to vesicoureteral reflux who underwent related-living donor kidney transplantation 2 years ago. Eleven months later, he was diagnosed with late antibody-mediated rejection. Despite an aggressive conventional immunosuppressive regimen, signs of rejection persisted. After the patient was treated with 2 doses of eculizumab, his mean fluorescence index dropped and serum creatinine decreased from 3.8 to 1.5 mg/dL. Case 2 was an unsensitized 16-year-old male patient with kidney failure secondary posterior urethral valve who underwent related-living donor kidney transplantation 4 years ago. Two years later, he was diagnosed with late antibody-mediated rejection. Despite an aggressive conventional immunosuppressive regimen, signs of rejection persisted. After treatment with 2 doses of eculizumab, his mean fluorescence index dropped and serum creatinine decreased from 2.1 to 1.01 mg/dL. In both patients, eculizumab therapy effectively reduced the markers of late antibody-mediated rejection and improved the kidney function.
迟发性抗体介导的排斥反应是导致肾移植失败的主要原因。肾移植受者中，针对迟发性抗体介导的排斥反应的有效治疗选择有限。在这里，我们报告了 2 例儿童严重迟发性抗体介导的排斥反应病例，这 2 例患者对常规免疫抑制治疗有抗性，使用依库珠单抗治疗后成功得到缓解。
本研究纳入了 2 名符合迟发性抗体介导的排斥反应诊断标准（供体特异性抗体阳性、平均荧光指数升高、微血管急性和/或慢性形态学损伤以及异常肾功能检查）的患者。这 2 名患者既往均无致敏且 panel-reactive antibody 阴性。
病例 1 为 12 岁男性，因膀胱输尿管反流导致肾衰竭，2 年前接受相关活体供肾移植。11 个月后，他被诊断为迟发性抗体介导的排斥反应。尽管采用了强化的常规免疫抑制治疗方案，但排斥反应仍持续存在。在接受 2 剂依库珠单抗治疗后，他的平均荧光指数下降，血清肌酐从 3.8mg/dL 降至 1.5mg/dL。
病例 2 为 16 岁男性，因后尿道瓣膜导致肾衰竭，4 年前接受相关活体供肾移植。2 年后，他被诊断为迟发性抗体介导的排斥反应。尽管采用了强化的常规免疫抑制治疗方案，但排斥反应仍持续存在。在接受 2 剂依库珠单抗治疗后，他的平均荧光指数下降，血清肌酐从 2.1mg/dL 降至 1.01mg/dL。
在这 2 例患者中，依库珠单抗治疗有效降低了迟发性抗体介导的排斥反应标志物，并改善了肾功能。
