Department of Pediatrics, Division of Cardiology, Medical University of South Carolina, Charleston, South Carolina, USA.
Department of Pediatrics, Division of Nephrology, Medical University of South Carolina, Charleston, South Carolina, USA.
J Clin Apher. 2022 Aug;37(4):411-414. doi: 10.1002/jca.21978. Epub 2022 Mar 3.
Low-density lipoprotein (LDL) apheresis has been shown to improve remission in patients with steroid-resistant nephrotic syndrome (SRNS). Here, we report a case study of two patients who failed apheresis treatment for SRNS and required transplant with subsequent recurrence of nephrotic syndrome and response to apheresis treatment. Two patients were treated with 12 sessions of LDL apheresis for SRNS without improvement and subsequently required renal transplantation. The first patient received an ABO-incompatible kidney transplant requiring plasma exchange (PE) with subsequent recurrence of focal segmental glomerulosclerosis. The second patient also received a renal transplant after treatment failure and subsequently developed recurrence of nephrotic syndrome in the transplanted kidney. Both patients underwent repeat therapy with lipoprotein apheresis. The first patient underwent lipoprotein apheresis after completing PE with significant improvement in serum creatinine and urine protein creatinine ratio. Three years later, he continued to do well and remains in remission. The second patient also responded well to repeat therapy with lipoprotein apheresis and had significant improvement with a urine protein creatinine ratio of 0.8 and a serum creatinine of 0.9 mg/dL 6 months after transplant. Lipoprotein apheresis was able to result in remission of nephrotic syndrome in these patients with posttransplant recurrence of disease. This is the first report of patients not responding to treatment pretransplant but responding posttransplant. Lipoprotein apheresis should be considered in patients with recurrence of nephrotic syndrome after renal transplantation even with a history of treatment failure prior to transplantation.
低密度脂蛋白(LDL)吸附已被证明可改善类固醇难治性肾病综合征(SRNS)患者的缓解率。在这里,我们报告了两例患者的病例研究,他们对 SRNS 的吸附治疗失败,需要进行移植,随后肾病综合征复发,并对吸附治疗有反应。两名患者因 SRNS 接受了 12 次 LDL 吸附治疗,但无改善,随后需要进行肾移植。第一例患者接受了 ABO 不相容的肾移植,需要进行血浆置换(PE),随后出现局灶节段性肾小球硬化复发。第二例患者在治疗失败后也接受了肾移植,随后移植肾脏出现肾病综合征复发。两名患者均接受了脂蛋白吸附治疗的重复治疗。第一例患者在完成 PE 后接受了脂蛋白吸附治疗,血清肌酐和尿蛋白肌酐比均显著改善。三年后,他的情况仍然良好,仍处于缓解状态。第二例患者也对重复的脂蛋白吸附治疗反应良好,移植后 6 个月尿蛋白肌酐比为 0.8,血清肌酐为 0.9mg/dL,有显著改善。脂蛋白吸附能够使这些疾病复发的移植后患者的肾病综合征得到缓解。这是首例报告的患者在移植前治疗无反应但移植后有反应的病例。即使在移植前有治疗失败史,脂蛋白吸附也应考虑用于移植后肾病综合征复发的患者。