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AA 淀粉样变性患者的肾移植:法国多中心队列研究的结果。

Kidney Transplantation in Patients With AA Amyloidosis: Outcomes in a French Multicenter Cohort.

机构信息

Sorbonne Université, Institut National de la Santé et de la Recherche Médicale, Unité Mixte de Recherche 1155, Soins Intensifs Néphrologiques et Rein Aigu, Département de Néphrologie, Assistance Publique-Hôpitaux de Paris, Hôpital Tenon, Paris, France; Université de Paris, Service de Néphrologie-Transplantation, Assistance Publique-Hôpitaux de Paris, Hôpital Bicêtre, Le Kremlin-Bicêtre, France.

Sorbonne Université, Internal Medicine Department, Assistance Publique-Hôpitaux de Paris, Hôpital Tenon, National Reference Center for Autoinflammatory Diseases and Inflammatory Amyloidosis, Groupe de recherche clinique Amylose AA Sorbonne Université (GRAASU), Paris, France.

出版信息

Am J Kidney Dis. 2024 Mar;83(3):329-339. doi: 10.1053/j.ajkd.2023.07.020. Epub 2023 Sep 22.

DOI:10.1053/j.ajkd.2023.07.020
PMID:37741608
Abstract

RATIONALE & OBJECTIVE: Outcomes of kidney transplantation for patients with renal AA amyloidosis are uncertain, with reports of poor survival and high rates of disease recurrence. However, the data are inconclusive and mostly based on studies from the early 2000s and earlier.

STUDY DESIGN

Retrospective multicenter cohort study.

SETTING & PARTICIPANTS: We searched the French national transplant database to identify all patients with renal AA amyloidosis who underwent kidney transplantation between 2008 and 2018.

EXPOSURES

Age, cause of amyloidosis, use of biotherapies, and C-reactive protein levels.

OUTCOMES

Outcomes were all-cause mortality and allograft loss. We also reported amyloidosis allograft recurrence, occurrence of acute rejection episodes, as well as infectious, cardiovascular, and neoplastic disease events.

ANALYTICAL APPROACH

Kaplan-Meier estimator for mortality and cumulative incidence function method for allograft loss. Factors associated with patient and allograft survival were investigated using a Cox proportional hazards model and a cause-specific hazards model, respectively.

RESULTS

86 patients who received kidney transplants for AA amyloidosis at 26 French centers were included. The median age was 49.4 years (IQR, 39.7-61.1). The main cause of amyloidosis was familial Mediterranean fever (37 cases; 43%). 16 (18.6%) patients received biotherapy after transplantation. Patient survival rates were 94.0% (95% CI, 89.1-99.2) at 1 year and 85.5% (77.8-94.0) at 5 years after transplantation. Cumulative incidences of allograft loss were 10.5% (4.0-17.0) at 1 year and 13.0% (5.8-20.1) at 5 years after transplantation. Histologically proven AA amyloidosis recurrence occurred in 5 transplants (5.8%). An infection requiring hospitalization developed in 55.8% of cases, and there was a 27.9% incidence of acute allograft rejection. Multivariable analysis showed that C-reactive protein concentration at the time of transplantation was associated with patient survival (HR, 1.01; 95% CI, 1.00-1.02; P=0.01) and allograft survival (HR, 1.68; 95% CI, 1.10-2.57; P=0.02).

LIMITATIONS

The study lacked a control group, and the effect of biotherapies on transplantation outcomes could not be explored.

CONCLUSIONS

This relatively contemporary cohort of patients who received a kidney transplant for AA amyloidosis experienced favorable rates of survival and lower recurrence rates than previously reported. These data support the practice of treating these patients with kidney transplantation for end-stage kidney disease.

PLAIN-LANGUAGE SUMMARY: AA amyloidosis is a severe and rare disease. Kidney involvement is frequent and leads to end-stage kidney disease. Because of the involvement of other organs, these patients are often frail, which has raised concerns about their suitability for kidney transplantation. We reviewed all patients with AA amyloidosis nephropathy who underwent kidney transplantation in France in the recent era (2008-2018) and found that the outcomes after kidney transplantation were favorable, with 85.5% of patients still alive 5 years after transplantation, a survival rate that is comparable to the outcomes of patients receiving a transplant for other forms of kidney diseases. Recurrence of amyloidosis in the transplanted kidney was infrequent (5.8%). These data support the practice of kidney transplantation for patients with AA amyloidosis who experience kidney failure.

摘要

背景与目的

肾 AA 淀粉样变性患者接受肾移植的结局不确定,有报道称其生存率低且疾病复发率高。然而,数据尚无定论,且主要基于 21 世纪初及更早的研究。

研究设计

回顾性多中心队列研究。

设置与参与者

我们检索了法国国家移植数据库,以确定所有在 2008 年至 2018 年间接受肾移植治疗的肾 AA 淀粉样变性患者。

暴露因素

年龄、淀粉样变性病因、生物治疗的使用以及 C 反应蛋白水平。

结局

全因死亡率和移植物丢失。我们还报告了淀粉样变性移植物复发、急性排斥反应发作的发生情况,以及感染、心血管和肿瘤疾病事件。

分析方法

使用 Kaplan-Meier 估计法进行死亡率分析,使用累积发生率函数法进行移植物丢失分析。使用 Cox 比例风险模型和特定原因风险模型分别研究与患者和移植物生存相关的因素。

结果

纳入了 26 家法国中心的 86 例接受 AA 淀粉样变性肾移植的患者。中位年龄为 49.4 岁(IQR,39.7-61.1)。淀粉样变性的主要病因是家族性地中海热(37 例;43%)。16 例(18.6%)患者在移植后接受了生物治疗。患者 1 年生存率为 94.0%(95%CI,89.1-99.2),5 年生存率为 85.5%(77.8-94.0)。移植物丢失的累积发生率在 1 年时为 10.5%(4.0-17.0),在 5 年时为 13.0%(5.8-20.1)。5 例(5.8%)移植后发生了组织学证实的 AA 淀粉样变性复发。55.8%的病例发生了需要住院治疗的感染,急性移植物排斥的发生率为 27.9%。多变量分析显示,移植时 C 反应蛋白浓度与患者生存(HR,1.01;95%CI,1.00-1.02;P=0.01)和移植物生存(HR,1.68;95%CI,1.10-2.57;P=0.02)相关。

局限性

该研究缺乏对照组,无法探讨生物治疗对移植结局的影响。

结论

本研究中,接受 AA 淀粉样变性肾移植的患者为较近时期的队列,其生存率较高,复发率较低,优于既往报道。这些数据支持对这些终末期肾病患者进行肾移植治疗的做法。

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