Xing Yan, Qin Yunlong, Li Xiayin, Wang Di, Zhao Jin, Zheng Wanting, Zhao Lijuan, Wu Hao, Sun Shiren
Department of Nephrology, Xijing Hospital, The Fourth Military Medical University, No.127 Changle West Road, Xi'an, Shaanxi Province, 710032, China.
Department of Nephrology, Bethune International Peace Hospital, No.398 Zhongshan West Road, Shijiazhuang, Hebei Province, 050051, China.
Int Urol Nephrol. 2025 Jan;57(1):275-283. doi: 10.1007/s11255-024-04182-7. Epub 2024 Aug 13.
Advancements in treatment regimens have led to improved outcomes in renal Immunoglobulin light-chain amyloidosis. Nevertheless, a subset of patients may still experience renal adverse events despite achieving hematologic very good partial response or better. This discrepancy may be attributed to the deposition pattern of amyloid in renal tissue. To enhance prognostic assessment, a staging system that incorporates both pathological characteristics and clinical indicators should be developed.
Patients newly diagnosed through renal biopsy between January 1, 2017, and December 31, 2022, were included. The renal pathology of patients was evaluated according to amyloid score (AS). Risk factors for end-stage renal disease or renal progression were identified by the competing risk model, then to develop a renal staging system. The Concordance index (C-index), internal cross-validation and Decision Curve Analysis (DCA) were used to evaluate the performance of the new staging system.
74 patients were included, and 16 (21.6%) patients had end-stage renal disease or renal progression within 24.7 (11.9, 50.7) months. AS and estimated glomerular filtration rate (eGFR) were identified as independent risk factors and the staging system based on them, which the C-index was 0.81 (95%CI, 0.73-0.89), had greater improvement than previous staging systems. The internal cross-validation and DCA also confirmed its great clinical benefits.
The AS demonstrated its prognostic significance in Chinese patients, and the novel renal staging system based on AS and eGFR may provide great prognostic guidance for these patients.
治疗方案的进步已使肾免疫球蛋白轻链淀粉样变性的治疗效果得到改善。然而，尽管部分患者已达到血液学非常好的部分缓解或更好的情况，但仍有一部分患者可能会出现肾脏不良事件。这种差异可能归因于淀粉样蛋白在肾组织中的沉积模式。为了加强预后评估，应开发一种结合病理特征和临床指标的分期系统。
纳入2017年1月1日至2022年12月31日期间通过肾活检新诊断的患者。根据淀粉样蛋白评分（AS）评估患者的肾脏病理。通过竞争风险模型确定终末期肾病或肾脏进展的危险因素，进而开发一种肾脏分期系统。采用一致性指数（C指数）、内部交叉验证和决策曲线分析（DCA）来评估新分期系统的性能。
共纳入74例患者，其中16例（21.6%）在24.7（11.9，50.7）个月内出现终末期肾病或肾脏进展。AS和估计肾小球滤过率（eGFR）被确定为独立危险因素，基于它们的分期系统的C指数为0.81（95%CI，0.73 - 0.89），比先前的分期系统有更大改进。内部交叉验证和DCA也证实了其巨大的临床益处。
AS在中国患者中显示出其预后意义，基于AS和eGFR的新型肾脏分期系统可能为这些患者提供重要的预后指导。
