A risk stratification for systemic immunoglobulin light‐chain amyloidosis with renal involvement

A risk stratification for systemic immunoglobulin light‐chain amyloidosis with renal involvement
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累及肾脏的全身免疫球蛋白轻链淀粉样变性的风险分层

DOI:
10.1111/bjh.16112
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发表时间:
2019
影响因子:
6.5
通讯作者:
Zhihong Liu
Zhihong Liu
中科院分区:
医学2区
文献类型:
--
作者:
Ting Li;Xianghua Huang;Qingwen Wang;Liang Zhao;G. Ren;Wencui Chen;C. Zheng;Minlin Zhou;Qi Jiang;Ru Yin;Zhihong Liu

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在约70%的系统性免疫球蛋白轻链(AL)淀粉样变性患者中发现肾脏受累。然而,目前尚无专门针对肾性AL患者生存率的风险分层系统。据报道,半乳糖凝集素-3(Gal-3)预示着其他肾脏疾病的预后不良。我们测量了253例连续诊断为肾性AL患者的基线样本中的Gal-3和几种传统的AL风险生物标志物。基线时,Gal-3 [风险比(HR):1·46; P = 0·033]、高敏心肌肌钙蛋白T(hs-cTnT)(HR:2.65; P <0.001)和受累和未受累游离轻链(dFLC)之间的差异(HR:1.81; P = 0.001)是全因死亡率的独立预测因素。Gal-3、hs-cTnT和dFLC的临界点分别为20·24 ng/ml、0·026 ng/ml和75·89 mg/l。通过对高于临界点的三种生物标志物中的每一种指定1分,将患者分为四个阶段。1、2、3、4期患者比例分别为17.0%、37.2%、29.2%、16.6%,中位生存期分别为100、60、29、15个月(P <0.01)。较高水平的Gal-3与死亡风险增加相关,基于Gal-3的风险分层是预测伴有肾脏受累的AL淀粉样变性死亡率的可靠模型。
Renal involvement is found in about 70% of patients with systemic immunoglobulin light‐chain (AL) amyloidosis. However, there is no risk stratification system specialized for renal AL concerning patients’ survival. Galectin‐3 (Gal‐3) has been reported to portend poor prognosis in other renal diseases. We measured Gal‐3 and several traditional risk biomarkers of AL in baseline samples from 253 consecutive patients diagnosed with renal AL. At baseline, Gal‐3 [Hazard ratio (HR): 1·46; P = 0·033], high‐sensitivity cardiac troponin T (hs‐cTnT) (HR: 2·65; P < 0·001) and difference between involved and uninvolved free light chains (dFLC) (HR: 1·81; P = 0·001) were independent predictors of all‐cause mortality. The cut‐off points for Gal‐3, hs‐cTnT, and dFLC were 20·24 ng/ml, 0·026 ng/ml, and 75·89 mg/l, respectively. Patients were stratified into four stages by assigning a score of 1 for each of the three biomarkers above the cut‐off point. The proportions of patients with disease stages 1, 2, 3 and 4 were 17·0%, 37·2%, 29·2% and 16·6%, and the median overall survival times from diagnosis were 100, 60, 29 and 15 months, respectively (P < 0·01). Higher level of Gal‐3 is associated with increased risk for mortality, and the risk stratification based on Gal‐3 is a reliable model for predicting mortality in AL amyloidosis with renal involvement.
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