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用ravulizumab治疗非典型溶血尿毒综合征的眼部表现:一例报告及文献综述

Ocular Manifestations in Atypical Hemolytic Uremic Syndrome Treated With Ravulizumab: A Case Report and Review of the Literature.

作者信息

Flindris Konstantinos, Papafotiou Eleni, Mylona Elena, Chatzipetrou Chrysa, Kaliardas Athanasios, Koumpoulis Ioannis, Melissourgos Ioannis

机构信息

Ophthalmology, General Hospital of Ioannina "G. Hatzikosta", Ioannina, GRC.

Medical School, Aristotle University of Thessaloniki, Thessaloniki, GRC.

出版信息

Cureus. 2025 May 17;17(5):e84271. doi: 10.7759/cureus.84271. eCollection 2025 May.

DOI:10.7759/cureus.84271
PMID:40530206
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC12170237/
Abstract

This study aims to report the ocular manifestations in a patient with atypical hemolytic uremic syndrome (aHUS), treated with ravulizumab, highlighting the reversibility of hypertensive retinal changes. We present a retrospective case report of a 27-year-old man diagnosed with aHUS, who underwent comprehensive systemic evaluation after a respiratory infection and a hypertensive crisis. Despite the absence of ophthalmologic complaints, ocular examination revealed grade IV hypertensive retinopathy accompanied by exudative retinal detachment. The patient received a combination of hemodialysis, intensive antihypertensive therapy, and ravulizumab. Remarkably, within one week of treatment initiation, a significant decrease in papilledema and a complete resolution of the exudative retinal detachment were observed. This case highlights the potential for rapid reversal of severe ocular manifestations associated with systemic microangiopathic disorders, such as aHUS. The striking improvement in retinal pathology suggests that the early and aggressive management, including complement inhibition with ravulizumab, may play a crucial role in restoring ocular integrity. Furthermore, the findings advocate for routine ophthalmologic screening in aHUS patients, even in the absence of visual symptoms, to enable prompt identification and intervention in subclinical retinal involvement. Interdisciplinary collaboration between nephrology and ophthalmology is essential to optimize both systemic and ocular outcomes in such complex cases.

摘要

本研究旨在报告1例接受ravulizumab治疗的非典型溶血尿毒综合征(aHUS)患者的眼部表现,强调高血压性视网膜病变的可逆性。我们报告1例27岁男性aHUS患者的回顾性病例,该患者在呼吸道感染和高血压危象后接受了全面的系统评估。尽管没有眼科主诉,但眼部检查发现IV级高血压性视网膜病变并伴有渗出性视网膜脱离。患者接受了血液透析、强化抗高血压治疗和ravulizumab联合治疗。值得注意的是,在开始治疗的1周内,视乳头水肿明显减轻,渗出性视网膜脱离完全消退。该病例突出了与系统性微血管病相关的严重眼部表现(如aHUS)迅速逆转的可能性。视网膜病变的显著改善表明,包括使用ravulizumab抑制补体在内的早期积极治疗可能在恢复眼部完整性方面发挥关键作用。此外,研究结果提倡对aHUS患者进行常规眼科筛查,即使没有视觉症状,以便及时发现并干预亚临床视网膜受累情况。在这种复杂病例中,肾脏病学和眼科之间的跨学科合作对于优化全身和眼部治疗效果至关重要。

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https://cdn.ncbi.nlm.nih.gov/pmc/blobs/facc/12170237/9a58f2213874/cureus-0017-00000084271-i08.jpg
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本文引用的文献

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Global aHUS Registry Analysis of Patients Switching to Ravulizumab From Eculizumab.从依库珠单抗转换为ravulizumab治疗的患者的全球非典型溶血尿毒症综合征注册研究分析
Kidney Int Rep. 2024 Jun 20;9(9):2648-2656. doi: 10.1016/j.ekir.2024.06.020. eCollection 2024 Sep.
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Clinical efficacy and safety of switching from eculizumab to ravulizumab in adult patients with aHUS- real-world data.依库珠单抗转换为瑞普昔韦单抗治疗成人抗 hUS 疗效和安全性:真实世界数据。
BMC Nephrol. 2024 Jun 19;25(1):202. doi: 10.1186/s12882-024-03638-3.
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Impact of Arterial Hypertension on the Eye: A Review of the Pathogenesis, Diagnostic Methods, and Treatment of Hypertensive Retinopathy.
高血压对眼睛的影响:高血压性视网膜病变的发病机制、诊断方法和治疗的综述。
Med Sci Monit. 2022 Jan 20;28:e935135. doi: 10.12659/MSM.935135.
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Ravulizumab for the Treatment of aHUS in Adults: Improving Quality of Life.ravulizumab用于治疗成人非典型溶血尿毒症综合征:改善生活质量。
Kidney Int Rep. 2021 May 12;6(6):1489-1491. doi: 10.1016/j.ekir.2021.04.036. eCollection 2021 Jun.
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Ravulizumab: A Review in Atypical Haemolytic Uraemic Syndrome.拉维珠单抗:治疗非典型溶血尿毒综合征的研究进展。
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Occlusive retinal vasculopathy with macular branch retinal artery occlusion as a leading sign of atypical hemolytic uremic syndrome - a case report.伴有黄斑分支视网膜动脉阻塞的闭塞性视网膜血管病变作为非典型溶血尿毒综合征的首要表现:一例报告。
BMC Ophthalmol. 2021 Jan 30;21(1):65. doi: 10.1186/s12886-021-01820-x.
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Kidney Int. 2020 Jun;97(6):1287-1296. doi: 10.1016/j.kint.2020.01.035. Epub 2020 Mar 6.
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Correction to: Eculizumab in atypical hemolytic uremic syndrome: strategies toward restrictive use.对《依库珠单抗治疗非典型溶血性尿毒症综合征:限制使用策略》的勘误
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