The Role of Kidney Transplantation in Monoclonal Ig Deposition Disease
The Role of Kidney Transplantation in Monoclonal Ig Deposition Disease
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DOI：
10.1016/j.ekir.2020.01.011
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发表时间：
2020-04-01
影响因子：
6
通讯作者：
Havasi，Andrea
中科院分区：
文献类型：
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作者：
Angel-Korman，Avital;Stern，Lauren;Havasi，Andrea
单克隆IG沉积病（MIDD）是一种常见的肾功能衰竭疾病，肾移植对这类患者有很大的益处。然而，在MIDD.MethodsThis是一个回顾性分析23例MIDD患者，包括6例接受肾transplant.ResultsThe 1-，5-，和10年的总生存率（OS）从诊断的长期肾脏预后的数据是有限的，分别为95%，78%和65%。大约一半的患者（n = 12）进展为终末期肾病（ESRD），从诊断到ESRD的中位时间为3.4年。诊断后1、5和10年肾存活率分别为77%、48%和29%。仅在5例患者（22%）中观察到肾脏缓解，所有患者均在达到血液学完全缓解后出现。接受肾移植的患者诊断后的中位OS显著优于维持透析的患者（19.8年vs. 8.3年，P= 0.016）。在接受肾移植的患者中，MIDD诊断的最短生存期为13.7年，最长为27.8年。在3例死亡的肾移植患者中，从首次肾移植到死亡的时间分别为7.4年、18.8年和20.4年。移植物丢失，由于疾病复发发生在4个月和3.8年后，肾移植的2例患者，无论是谁没有治疗或没有回应treatment.ConclusionAs治疗MIDD显着改善，更多的患者实现持续的血液反应，更长的患者和移植肾移植后的生存。
IntroductionMonoclonal Ig deposition disease (MIDD) frequently leads to kidney failure, and a large proportion of these patients would greatly benefit from kidney transplantation. However, data on kidney transplantation outcomes in MIDD are limited.MethodsThis was a retrospective analysis of long-term renal outcomes of 23 patients with MIDD, including 6 patients who underwent kidney transplantation.ResultsThe 1-, 5-, and 10-year overall survival (OS) from diagnosis were 95%, 78%, and 65%, respectively. Approximately half of the patients (n = 12) progressed to end-stage renal disease (ESRD) with a median time from diagnosis to ESRD of 3.4 years. The 1-, 5-, and 10-year renal survival from diagnosis were 77%, 48%, and 29% respectively. Renal response was observed only in 5 patients (22%), all of them after achieving hematologic complete response. Median OS from diagnosis was significantly better for those who underwent kidney transplantation versus those who remained on dialysis (19.8 years vs. 8.3 years,P= 0.016). Among patients who underwent kidney transplantation, the shortest survival from MIDD diagnosis was 13.7 years and the longest was 27.8 years. Of the 3 patients with kidney transplants who died, the time from the first kidney transplantation to death was 7.4, 18.8, and 20.4 years. Graft loss due to disease recurrence occurred at 4 months and 3.8 years after kidney transplantation in 2 patients who either were not treated or did not respond to treatment.ConclusionAs treatments for MIDD have dramatically improved, more patients are achieving sustained hematologic responses with longer patient and graft survival after kidney transplantation.