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依库珠单抗在继发性非典型溶血性尿毒症综合征中的合理应用。

Rational use of eculizumab in secondary atypical hemolytic uremic syndrome.

作者信息

Cordero Lucía, Cavero Teresa, Gutiérrez Eduardo, Trujillo Hernando, Sandino Justo, Auñón Pilar, Rivero Marta, Morales Enrique

机构信息

Nephrology Department, Hospital Universitario 12 de Octubre, Madrid, Spain.

Instituto de Investigación, Hospital Universitario 12 de Octubre (imas12), Madrid, Spain.

出版信息

Front Immunol. 2024 Jan 11;14:1310469. doi: 10.3389/fimmu.2023.1310469. eCollection 2023.


DOI:10.3389/fimmu.2023.1310469
PMID:38274833
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC10808527/
Abstract

BACKGROUND: Secondary atypical hemolytic uremic syndrome (secondary aHUS) is a heterogeneous group of thrombotic microangiopathies (TMA) associated with various underlying conditions. Unlike primary aHUS, there is still no hard evidence on the efficacy of complement blockade in secondary aHUS, since the two main series that investigated this subject showed discrepant results. Our work aims to reassess the efficacy of eculizumab in treating secondary aHUS. METHODS: Observational, retrospective, single-center study, in which we analyzed the hematological and renal evolution of 23 patients diagnosed with secondary aHUS who received treatment with eculizumab and compared them with a control cohort of 14 patients. Complete renal response was defined as the recovery of renal function before the event, partial renal response as a recovery of 50% of lost glomerular filtration rate, and hematological response as normalization of hemoglobin and platelets. RESULTS: We found no statistically significant differences in baseline characteristics or disease severity between both groups. After a median of 5 doses of eculizumab, the group of patients who received complement blockade presented a significant difference in renal response (complete in 52.3% of patients and partial in 23.8%) compared to the control cohort (complete response 14.3% and partial of 14.3%). Rates of hematological remission were similar in both groups (90.9% in the eculizumab cohort and 85.7% in the control cohort). CONCLUSION: Early and short-term use of eculizumab in patients with secondary aHUS could be an effective and safe therapeutic option, assuring better renal recovery compared to patients who do not receive complement blockade.

摘要

背景:继发性非典型溶血性尿毒症综合征(继发性aHUS)是一组与各种潜在疾病相关的血栓性微血管病(TMA)的异质性疾病。与原发性aHUS不同,由于两项主要研究该主题的系列研究结果存在差异,目前仍没有确凿证据证明补体阻断在继发性aHUS中的疗效。我们的研究旨在重新评估依库珠单抗治疗继发性aHUS的疗效。 方法:进行观察性、回顾性、单中心研究,分析23例诊断为继发性aHUS并接受依库珠单抗治疗的患者的血液学和肾脏情况演变,并与14例患者的对照队列进行比较。完全肾脏缓解定义为事件发生前肾功能恢复,部分肾脏缓解定义为丢失的肾小球滤过率恢复50%,血液学缓解定义为血红蛋白和血小板恢复正常。 结果:我们发现两组患者的基线特征或疾病严重程度没有统计学上的显著差异。在接受中位数为5剂依库珠单抗治疗后,接受补体阻断的患者组在肾脏反应方面存在显著差异(52.3%的患者完全缓解,23.8%的患者部分缓解),而对照队列的完全缓解率为14.3%,部分缓解率为14.3%。两组的血液学缓解率相似(依库珠单抗队列中为90.9%,对照队列中为85.7%)。 结论:对于继发性aHUS患者,早期和短期使用依库珠单抗可能是一种有效且安全的治疗选择,与未接受补体阻断的患者相比,可确保更好的肾脏恢复。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7d8d/10808527/41fd61a940ed/fimmu-14-1310469-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7d8d/10808527/41fd61a940ed/fimmu-14-1310469-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7d8d/10808527/41fd61a940ed/fimmu-14-1310469-g001.jpg

相似文献

[1]
Rational use of eculizumab in secondary atypical hemolytic uremic syndrome.

Front Immunol. 2024-1-11

[2]
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[3]
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[4]
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[5]
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[6]
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[7]
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[8]
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[9]
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[10]
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引用本文的文献

[1]
Influenza A H1N1 Triggering Complement-Mediated Hemolytic Uremic Syndrome.

J Med Cases. 2025-7-19

[2]
Hypertensive Emergency and Atypical Hemolytic Uremic Syndrome Associated with Cocaine Use: A Diagnostic and Therapeutic Challenge.

Diseases. 2025-5-15

[3]
Atypical Hemolytic Uremic Syndrome Associated with BNT162b2 mRNA COVID-19 Vaccine in a Kidney Transplant Recipient: A Case Report and Literature Review.

Infect Dis Rep. 2025-2-11

[4]
Targeted Complement Treatments in Glomerulopathies: A Comprehensive Review.

J Clin Med. 2025-1-22

本文引用的文献

[1]
Epidemiology, Outcomes, and Complement Gene Variants in Secondary Thrombotic Microangiopathies.

Clin J Am Soc Nephrol. 2023-7-1

[2]
How I diagnose and treat atypical hemolytic uremic syndrome.

Blood. 2023-3-2

[3]
Complement Factor H-Related Proteins FHR1 and FHR5 Interact With Extracellular Matrix Ligands, Reduce Factor H Regulatory Activity and Enhance Complement Activation.

Front Immunol. 2022

[4]
Application of C5 inhibitors in glomerular diseases in 2021.

Kidney Res Clin Pract. 2022-7

[5]
Monoclonal Gammopathy of Renal Significance.

N Engl J Med. 2021-5-20

[6]
Eculizumab and aHUS: to stop or not.

Blood. 2021-5-6

[7]
Thrombotic microangiopathy in aHUS and beyond: clinical clues from complement genetics.

Nat Rev Nephrol. 2021-8

[8]
Functional and Genetic Landscape of Complement Dysregulation Along the Spectrum of Thrombotic Microangiopathy and its Potential Implications on Clinical Outcomes.

Kidney Int Rep. 2021-2-3

[9]
The long-acting C5 inhibitor, ravulizumab, is effective and safe in pediatric patients with atypical hemolytic uremic syndrome naïve to complement inhibitor treatment.

Kidney Int. 2021-7

[10]
Complement in Secondary Thrombotic Microangiopathy.

Kidney Int Rep. 2021-1

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