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重症自身炎症性疾病（SAVI）的眼部并发症：一例双侧葡萄膜炎和视网膜血管炎的独特病例。

Ocular complications of SAVI: A unique case of bilateral uveitis and retinal vasculitis.

作者信息

Menean Matteo, Marchese Alessandro, Modorati Giulio, Favale Rosa, Boscia Francesco, Bandello Francesco, Miserocchi Elisabetta

机构信息

Division of Head and Neck, Ophthalmology Unit, IRCCS San Raffaele Scientific Institute, Milan, Italy.

School of Medicine, Vita-Salute San Raffaele University, Milan, Italy.

出版信息

Am J Ophthalmol Case Rep. 2024 Apr 10;34:102046. doi: 10.1016/j.ajoc.2024.102046. eCollection 2024 Jun.

DOI:10.1016/j.ajoc.2024.102046
PMID:38633003
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11021826/
Abstract

PURPOSE

To describe a case of bilateral panuveitis in a patient with Stimulator of Interferon Genes (STING)-Associated Vasculitis with Onset in Infancy (SAVI).

OBSERVATIONS

A 45-year-old patient diagnosed with SAVI presented bilateral panuveitis and uncontrolled secondary intraocular hypertension due to structural complications from uveitis. Multimodal imaging revealed the presence of intraretinal fluid and bilateral vasculitis. The patient was started with systemic methotrexate.

CONCLUSIONS AND IMPORTANCE

This case is essential to characterize ocular involvement in patients with SAVI. Awareness of these ocular manifestations is crucial for timely management and improvement of visual prognosis.

摘要

目的

描述1例患有婴儿期起病的干扰素基因刺激物（STING）相关血管炎（SAVI）患者的双侧全葡萄膜炎。

观察结果

一名被诊断为SAVI的45岁患者出现双侧全葡萄膜炎，由于葡萄膜炎的结构并发症导致继发性眼压控制不佳。多模式成像显示存在视网膜内液和双侧血管炎。患者开始接受全身甲氨蝶呤治疗。

结论与意义

该病例对于明确SAVI患者的眼部受累情况至关重要。认识这些眼部表现对于及时治疗和改善视觉预后至关重要。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2c0e/11021826/eab3663fcb47/gr3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2c0e/11021826/426aef5c7249/gr1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2c0e/11021826/efe732e48af4/gr2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2c0e/11021826/eab3663fcb47/gr3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2c0e/11021826/426aef5c7249/gr1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2c0e/11021826/efe732e48af4/gr2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2c0e/11021826/eab3663fcb47/gr3.jpg

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本文引用的文献

1
The 2021 European Alliance of Associations for Rheumatology/American College of Rheumatology points to consider for diagnosis and management of autoinflammatory type I interferonopathies: CANDLE/PRAAS, SAVI and AGS.2021 年欧洲风湿病学会联盟/美国风湿病学会关于自身炎症性 I 型干扰素病的诊断和治疗的考虑要点：CANDLE/PRAAS、SAVI 和 AGS。
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Retinal vasculopathy in STING-associated vasculitis of infancy (SAVI).婴儿期STING相关血管炎（SAVI）中的视网膜血管病变
Rheumatology (Oxford). 2021 Oct 2;60(10):e351-e353. doi: 10.1093/rheumatology/keab297.
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Stimulator of Interferon Genes-Associated Vasculopathy With Onset in Infancy: A Systematic Review of Case Reports.
婴儿期起病的干扰素基因刺激因子相关血管病：病例报告的系统评价
Front Pediatr. 2020 Dec 17;8:577918. doi: 10.3389/fped.2020.577918. eCollection 2020.
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Overview of STING-Associated Vasculopathy with Onset in Infancy (SAVI) Among 21 Patients.21 例婴儿起病的 STING 相关性血管病（SAVI）概述。
J Allergy Clin Immunol Pract. 2021 Feb;9(2):803-818.e11. doi: 10.1016/j.jaip.2020.11.007. Epub 2020 Nov 18.
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Adult-Onset ANCA-Associated Vasculitis in SAVI: Extension of the Phenotypic Spectrum, Case Report and Review of the Literature.成人起病的 ANCA 相关性血管炎在 SAVI 中的表现：表型谱的扩展，病例报告及文献复习。
Front Immunol. 2020 Sep 29;11:575219. doi: 10.3389/fimmu.2020.575219. eCollection 2020.
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C-terminal truncations in human 3'-5' DNA exonuclease TREX1 cause autosomal dominant retinal vasculopathy with cerebral leukodystrophy.人类3'-5' DNA核酸外切酶TREX1的C末端截短会导致伴有脑白质营养不良的常染色体显性视网膜血管病变。
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