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伴有肾脏受累的轻链淀粉样变性:中国人群的肾脏结局和两种肾脏分期系统的验证。

Light-chain amyloidosis with renal involvement: renal outcomes and validation of two renal staging systems in the Chinese population.

机构信息

Department of Nephrology, Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medical Sciences , Beijing , China.

出版信息

Amyloid. 2019 Dec;26(4):186-191. doi: 10.1080/13506129.2019.1639149. Epub 2019 Jul 24.

DOI:10.1080/13506129.2019.1639149
PMID:31339366
Abstract

Renal involvement is one of the most common complications of light-chain (AL) amyloidosis. For evaluating renal prognosis, two staging systems for renal involvement have been proposed, one in 2014 and one in 2017. However, the two staging systems have not yet been compared and widely used in clinic. A total of 76 patients with newly diagnosed AL amyloidosis and renal involvement proven by renal biopsy were included and followed up with an endpoint developing to dialysis. The renal outcome and two criteria were explored. We confirmed the prognostic value of the 2014 renal staging system based on estimated glomerular filtration rate (eGFR) (<50 ml/min/1.73 m) and proteinuria (>5 g/day) at diagnosis ( = 0.003). For the 2017 system, none of the patients progressed to dialysis in both stage 1 (24 h proteinuria to eGFR <30 mg/ml/min/1.73 m) and stage 2 (24 h proteinuria to eGFR 30-99 mg/ml/min/1.73 m). A significant difference in terms of requiring dialysis was seen only between stage 3 (24 h proteinuria to eGFR ≥100 mg/ml/min/1.73 m) and the two other stages ( = 0.008). The prognostic value of the criteria based on eGFR and 24-hour proteinuria for predicting dialysis has been confirmed. These results might benefit guiding clinical treatment.

摘要

肾脏受累是轻链(AL)淀粉样变性最常见的并发症之一。为了评估肾脏预后,已经提出了两种用于肾脏受累的分期系统,一种是在 2014 年,另一种是在 2017 年。然而,这两种分期系统尚未进行比较,也尚未在临床上广泛应用。

共纳入 76 例经肾活检证实为新发 AL 淀粉样变性且伴有肾脏受累的患者,并随访至进展为透析的终点。探讨了肾脏结局和两种标准。

我们证实了基于估计肾小球滤过率(eGFR)(<50 ml/min/1.73 m)和诊断时蛋白尿(>5 g/天)的 2014 年肾脏分期系统的预后价值( = 0.003)。对于 2017 年系统,在 1 期(24 小时蛋白尿至 eGFR <30 mg/ml/min/1.73 m)和 2 期(24 小时蛋白尿至 eGFR 30-99 mg/ml/min/1.73 m),没有患者进展到透析。仅在 3 期(24 小时蛋白尿至 eGFR ≥100 mg/ml/min/1.73 m)与其他两个分期之间( = 0.008)观察到在需要透析方面存在显著差异。

基于 eGFR 和 24 小时蛋白尿的标准预测透析的预后价值已得到证实。这些结果可能有助于指导临床治疗。

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A prognostic staging system for light-chain amyloidosis using hepatic and renal indicator data from 1,064 Chinese patients.
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