The importance of kidney response over hematologic response in predicting kidney outcome in amyloid light-chain amyloidosis.

IF 2.9 3区医学 Q1 UROLOGY & NEPHROLOGY

Kidney Research and Clinical Practice Pub Date : 2024-12-05 DOI:10.23876/j.krcp.24.111

Sungmi Kim, Jinyoung Yang, Kyungho Lee, Junseok Jeon, Sang Eun Yoon, Darae Kim, Jin-Oh Choi, Seok Jin Kim, Kihyun Kim, Jung Eun Lee

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Abstract

Background: Light chain amyloidosis, characterized by amyloid fibril deposition in multiple organs, often leads to progression to endstage kidney disease. This study aimed to identify predictors of kidney survival in patients with kidney amyloidosis, focusing on hematologic and kidney response.

Methods: This retrospective study included 138 patients diagnosed with kidney amyloidosis between 2011 and 2019. Palladini criteria were applied to categorize kidney stage and kidney response based on initial glomerular filtration rate and proteinuria, and their changes after treatment. Hematologic response was assessed based on the 2012 International Society of Amyloidosis criteria. Deep hematologic response was defined as the achievement of at least a very good partial response.

Results: Overall, 17 (12.3%) progressed to end-stage kidney disease. Multivariable analysis, considering baseline characteristics, revealed that stage II had an increased risk of end-stage kidney disease compared to stage I (hazard ratio, 3.75; 95% confidence interval [CI], 1.38-10.15; p = 0.01). Compared to kidney response, the risk of end-stage kidney disease increased by 8.42 (95% CI, 1.72-41.35; p = 0.01) and 7.36 (95% CI, 1.25-43.33; p = 0.03) times in stable disease and kidney progression at 6 months, respectively, whereas deep hematologic response showed no association with kidney outcome. Kidney survival was longer in patients with deep hematologic response and kidney response than in those with only hematologic response (p = 0.004).

Conclusion: The study underscores the importance of kidney response over hematologic response in predicting end-stage kidney disease and emphasizes the need to assess treatment endpoints, considering organ response alongside hematologic response.

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在预测淀粉样蛋白轻链淀粉样变性患者肾脏预后时，肾脏反应比血液反应的重要性。

背景：以淀粉样纤维沉积在多个器官为特征的轻链淀粉样变性常导致进展为终末期肾脏疾病。本研究旨在确定肾脏淀粉样变患者肾脏生存的预测因素，重点关注血液学和肾脏反应。方法：本回顾性研究纳入了2011年至2019年诊断为肾脏淀粉样变的138例患者。根据初始肾小球滤过率和蛋白尿及其治疗后的变化，采用Palladini标准对肾脏分期和肾脏反应进行分类。血液学反应是根据2012年国际淀粉样变性协会的标准进行评估的。深度血液学反应被定义为至少达到非常好的部分反应。结果：总体而言，17例（12.3%）进展为终末期肾病。考虑到基线特征的多变量分析显示，II期患者发生终末期肾脏疾病的风险高于I期患者(风险比，3.75；95%置信区间[CI]， 1.38-10.15；P = 0.01)。与肾脏反应相比，终末期肾脏疾病的风险增加了8.42 (95% CI, 1.72-41.35；p = 0.01)和7.36 (95% CI, 1.25-43.33；P = 0.03)，而深度血液学反应与肾脏预后无相关性。有深度血液学反应和肾脏反应的患者的肾脏生存期长于只有血液学反应的患者（p = 0.004）。结论：该研究强调了肾脏反应比血液反应在预测终末期肾脏疾病中的重要性，并强调了评估治疗终点的必要性，同时考虑器官反应和血液反应。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Kidney Research and Clinical Practice UROLOGY & NEPHROLOGY-

CiteScore

4.60

自引率

10.00%

发文量

审稿时长

10 weeks

期刊介绍： Kidney Research and Clinical Practice (formerly The Korean Journal of Nephrology; ISSN 1975-9460, launched in 1982), the official journal of the Korean Society of Nephrology, is an international, peer-reviewed journal published in English. Its ISO abbreviation is Kidney Res Clin Pract. To provide an efficient venue for dissemination of knowledge and discussion of topics related to basic renal science and clinical practice, the journal offers open access (free submission and free access) and considers articles on all aspects of clinical nephrology and hypertension as well as related molecular genetics, anatomy, pathology, physiology, pharmacology, and immunology. In particular, the journal focuses on translational renal research that helps bridging laboratory discovery with the diagnosis and treatment of human kidney disease. Topics covered include basic science with possible clinical applicability and papers on the pathophysiological basis of disease processes of the kidney. Original researches from areas of intervention nephrology or dialysis access are also welcomed. Major article types considered for publication include original research and reviews on current topics of interest. Accepted manuscripts are granted free online open-access immediately after publication, which permits its users to read, download, copy, distribute, print, search, or link to the full texts of its articles to facilitate access to a broad readership. Circulation number of print copies is 1,600.