骨髓增生异常综合征的肾脏受累。

IF 3.9 2区 医学 Q1 UROLOGY & NEPHROLOGY Clinical Kidney Journal Pub Date : 2024-06-19 eCollection Date: 2024-08-01 DOI:10.1093/ckj/sfae185
Marie-Camille Lafargue, Jean-Paul Duong Van Huyen, Helmut G Rennke, Marie Essig, Mickaël Bobot, Noémie Jourde-Chiche, Hamza Sakhi, Alexandre Karras, Idris Boudhabhay, Philippe Brunet, Hugoline Boulay, Vincent Grobost, Carole Philipponnet, Juliette Jeannel, Jonathan Chemouny, Jean-Jacques Boffa, Dorra Braham-Stambouli, Umut Selamet, Leonardo V Riella, Olivier Fain, Lionel Adès, Pierre Fenaux, Camille Cohen, Arsène Mekinian
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引用次数: 0

摘要

简介:本研究旨在描述骨髓增生异常综合征(MDS)患者的肾脏受累情况:本研究旨在描述骨髓增生异常综合征(MDS)患者的肾脏受累情况、治疗方法和结果:我们在七个中心开展了一项多中心回顾性研究,确定了急性肾损伤(AKI)、慢性肾病(CKD)和尿液异常的MDS患者:15名患者在确诊MDS 3个月后出现肾脏疾病。尿蛋白与肌酐比值中位数为 1.9 g/g,血清肌酐中位数为 3.2 mg/dL。10 名患者在发病时出现了 AKI,12 名患者出现了肾脏以外的症状。肾脏诊断包括抗中性粒细胞胞浆抗体(ANCA)相关性血管炎(AAV)、ANCA 阴性血管炎、C3 肾小球肾炎、免疫复合物介导的肾小球肾炎、结节性多动脉炎和 IgA 血管炎。除一人外,所有患者都接受了针对 MDS 相关肾损伤的特殊治疗。在接受阿扎胞苷治疗的 6 名患者中,可以评估 MDS 治疗对肾损伤的影响,但肾功能的变化各不相同。中位随访 14 个月后,4 名患者进入 CKD 3 期,5 名患者进入 CKD 4 期,3 名患者进入终末期肾病。另一方面,3 名患者发展为急性髓性白血病,3 名患者死亡。与84名MDS对照组患者相比,肾脏受累的患者更年轻,发育不良的系数更多,更有资格接受低甲基化药物治疗,但两组患者的存活率没有差异。与265例未患MDS的AAV患者相比,10例患有MDS相关的贫免疫性血管炎的患者年龄更大,ANCA血清学更常呈阴性,皮肤病变更多:结论:与MDS相关的肾损伤主要表现为肾小球受累的血管炎,尤其是AAV。
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Kidney involvement in myelodysplastic syndromes.

Introduction: The objective of this study was to describe kidney involvement in patients with myelodysplastic syndromes (MDS), their treatments, and outcomes.

Methods: We conducted a multicenter retrospective study in seven centers, identifying MDS patients with acute kidney injury (AKI), chronic kidney disease (CKD), and urine abnormalities.

Results: Fifteen patients developed a kidney disease 3 months after MDS diagnosis. Median urine protein-to-creatinine ratio was 1.9 g/g, and median serum creatinine was 3.2 mg/dL. Ten patients had AKI at presentation, and 12 had extra-renal symptoms. The renal diagnoses included anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV), ANCA negative vasculitis, C3 glomerulonephritis, immune complex-mediated glomerulonephritis, polyarteritis nodosa, and IgA vasculitis. All patients but one received a specific treatment for the MDS-associated kidney injury. The effect of MDS treatment on kidney injury could be assessed in six patients treated with azacitidine, and renal function evolution was heterogenous. After a median follow-up of 14 months, four patients had CKD stage 3, five had CKD stage 4, and three had end stage kidney disease. On the other hand, three evolved to an acute myeloid leukemia and three died. Compared to 84 MDS controls, patients who had kidney involvement were younger, had a higher number of dysplasia lineages, and were more eligible to receive hypomethylating agents, but no survival difference was seen between the two groups. Compared to 265 AAV without MDS, the ten with MDS-associated pauci-immune vasculitis were older, ANCA serology was more frequently negative, and more cutaneous lesions were seen.

Conclusion: The spectrum of kidney injuries associated with MDS is mostly represented by vasculitis with glomerular involvement, and especially AAV.

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来源期刊
Clinical Kidney Journal
Clinical Kidney Journal Medicine-Transplantation
CiteScore
6.70
自引率
10.90%
发文量
242
审稿时长
8 weeks
期刊介绍: About the Journal Clinical Kidney Journal: Clinical and Translational Nephrology (ckj), an official journal of the ERA-EDTA (European Renal Association-European Dialysis and Transplant Association), is a fully open access, online only journal publishing bimonthly. The journal is an essential educational and training resource integrating clinical, translational and educational research into clinical practice. ckj aims to contribute to a translational research culture among nephrologists and kidney pathologists that helps close the gap between basic researchers and practicing clinicians and promote sorely needed innovation in the Nephrology field. All research articles in this journal have undergone peer review.
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