Progress in Retinal and Eye Research最新文献

Quantification of retinal microvascular imaging features from fundus photos in ocular and systemic disease: a framework for standardization 眼部和全身性疾病眼底照片中视网膜微血管成像特征的量化：标准化框架

IF 17.8 1区医学 Q1 OPHTHALMOLOGY

Progress in Retinal and Eye Research

Pub Date : 2026-03-21 DOI: 10.1016/j.preteyeres.2026.101461

Frank C.T. van der Heide, Youba Zamoum, Mehdi Ounissi, Tos TJM Berendschot, Carol Y. Cheung, Maya Koronyo-Hamaoui, Leopold Schmetterer, Jacqueline Chua, Tunde Peto, Imre Lengyel, Lajos Csincsik, Catherine Creuzot, Louis Arnould, Ingeborg Stalmans, Emanuele Trucco, Tom Macgillivray, Anthony P. Khawaja, Lisa Zhuoting Zhu, Pearse A. Keane, Tien Yin Wong, Dan Milea

引用次数: 0

Inner-retinal changes in AMD: Evidence, mechanisms, and future perspectives 黄斑变性视网膜内改变：证据、机制和未来展望

IF 17.8 1区医学 Q1 OPHTHALMOLOGY

Progress in Retinal and Eye Research

Pub Date : 2026-03-21 DOI: 10.1016/j.preteyeres.2026.101463

Matt Trinh, Michael Kalloniatis, Bryan William Jones, Glenn C. Yiu, Enrico Borrelli, Lisa Nivison-Smith

引用次数: 0

The Role of Prostanoids in the Retina and Optic Nerve in Health and Disease. 前列腺素在视网膜和视神经健康和疾病中的作用。

IF 14.7 1区医学 Q1 OPHTHALMOLOGY

Progress in Retinal and Eye Research

Pub Date : 2026-03-17 DOI: 10.1016/j.preteyeres.2026.101460

Emily Ward, Shruthi Karnam, Amogh Changavi, Matangi Kumar, Shubham Maurya, Milica A Margeta, Jeremy M Sivak, Karsten Gronert, John G Flanagan

Prostaglandin D₂ (PGD₂), PGE₂, and other prostanoids play important roles in retinal and optic nerve (ON) health and disease, including neovascularization, neuroinflammation, and neurodegeneration. We reviewed the synthesis, expression, regulation, mechanisms, and functions of the principal prostanoids (PGF₂α, PGI₂, TXA₂, PGE₂, and PGD₂) in the eye. Prostanoids can be difficult to study in-vivo or ex-vivo because they are small, unstable molecules. To address this, we incorporated lipidomics data clarifying the prostanoids present in the mouse retina and ON, and mined single-cell transcriptomic atlases of the human and mouse posterior eye to map prostanoid pathway expression across cell types. We identified species-, tissue-, and region-specific differences in prostanoid synthase and receptor expression. The PGF₂α receptor (FP) was detected in mouse but not human retinal ganglion cells (RGCs). TXA₂ synthase was present in ON but not retinal glia. DP2 and EP1 were enriched in microglia and astrocytes of the retrolaminar ON, but not the ON head. Analysis of mouse RGC degeneration datasets revealed dynamic regulation of prostanoid pathways in glia and RGCs. During early degeneration, macroglia upregulated PGD₂ synthase (L-PGDS), while microglia upregulated the PGE₂ receptor EP4. Glia downregulated intrinsic COX-1 pathways, yet we did not observe induction of COX-2. Topical NSAID treatment exacerbates RGC damage following mouse ocular hypertension, underscoring the need to clarify COX-1/2 roles in the eye. In conclusion, intrinsic prostanoid networks support retinal and ON homeostasis and are dynamically regulated in disease, representing cell-specific, receptor, and disease stage therapeutic targets, including potential RGC protection.

前列腺素D2 （PGD2）、PGE2和其他类前列腺素在视网膜和视神经（ON）健康和疾病中发挥重要作用，包括新生血管、神经炎症和神经变性。本文综述了眼内主要前列腺素（PGF2α、PGI2、TXA2、PGE2和PGD2）的合成、表达、调控、机制和功能。类前列腺素很难在体内或离体研究，因为它们是小而不稳定的分子。为了解决这个问题，我们结合了脂质组学数据，阐明了小鼠视网膜和ON中存在的前列腺素，并挖掘了人类和小鼠后眼的单细胞转录组图谱，以绘制不同细胞类型的前列腺素通路表达。我们确定了前列腺素合成酶和受体表达的物种、组织和区域特异性差异。PGF2α受体（FP）在小鼠视网膜神经节细胞（RGCs）中检测到，而在人视网膜神经节细胞（RGCs）中检测不到。视网膜胶质细胞中不存在TXA2合成酶。膜后ON的小胶质细胞和星形胶质细胞中富集DP2和EP1，而ON头部中不富集。对小鼠RGC变性数据集的分析揭示了胶质细胞和RGC中前列腺素通路的动态调节。在早期变性过程中，大胶质细胞上调PGD2合成酶（L-PGDS），而小胶质细胞上调PGE2受体EP4。胶质细胞下调了内在的COX-1通路，但我们没有观察到COX-2的诱导。局部非甾体抗炎药治疗会加重小鼠高眼压后的RGC损伤，因此需要明确COX-1/2在眼睛中的作用。总之，内在前列腺素网络支持视网膜和ON稳态，并在疾病中受到动态调节，代表细胞特异性、受体和疾病阶段的治疗靶点，包括潜在的RGC保护。

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引用次数: 0

Outer retinal tubulation associated with photoreceptor degeneration 视网膜外管化与光感受器变性有关

IF 14.7 1区医学 Q1 OPHTHALMOLOGY

Progress in Retinal and Eye Research

Pub Date : 2026-03-01 Epub Date: 2026-01-16 DOI: 10.1016/j.preteyeres.2026.101435

Victor Lin , Winston Lee , Eugene Yu-Chuan Kang , Pei-Kang Liu , Nan-Kai Wang

Outer retinal tubulation (ORT) is a distinct structural manifestation of chronic photoreceptor degeneration, observed across a broad spectrum of retinal diseases. Initially described histologically as rosette-like formations, ORT has gained clinical relevance with the advent of high-resolution imaging modalities such as spectral-domain optical coherence tomography (SD-OCT) and adaptive optics scanning laser ophthalmoscopy (AO-SLO), which enable in vivo visualization of its tubular architecture. ORT arises from sustained photoreceptor and retinal pigment epithelium (RPE) injury, leading to the reorganization of surviving cones ensheathed by gliotic Müller cell processes. This review integrates historical, histological, and imaging data to elucidate ORT's cellular composition, formation mechanisms, and disease-specific patterns. We introduce a novel etiological classification of ORT, categorized as degenerative, fibrotic, or edematous ORT according to predominant pathogenic drivers, to facilitate cross-disease comparison and prognostic stratification. Clinically, ORT serves as a non-exudative biomarker of chronic retinal injury, aiding differential diagnosis and informing treatment strategies. In age-related macular degeneration, ORT is associated with subretinal fibrosis and poor visual outcomes; in geographic atrophy, it may signal slower lesion progression. In inherited retinal dystrophies, ORT reflects genotype-specific vulnerabilities and residual photoreceptor survival, with implications for therapeutic targeting. As imaging technologies advance, ORT offers promise as a structural marker of disease chronicity, photoreceptor resilience, and Müller cell plasticity, enhancing diagnostic precision and supporting its role as a meaningful endpoint in clinical trials.

视网膜外管化（ORT）是慢性光感受器变性的一种独特的结构表现，在广泛的视网膜疾病中观察到。ORT最初在组织学上被描述为玫瑰状结构，随着高分辨率成像方式的出现，如光谱域光学相干断层扫描（SD-OCT）和自适应光学扫描激光检眼镜（AO-SLO）的出现，ORT获得了临床意义，这些成像方式能够在体内可视化其管状结构。ORT是由持续的光感受器和视网膜色素上皮（RPE）损伤引起的，导致被胶质细胞勒细胞突包裹的幸存视锥细胞重组。这篇综述整合了历史、组织学和影像学数据来阐明ORT的细胞组成、形成机制和疾病特异性模式。我们介绍了一种新的ORT病因学分类，根据主要的致病因素将ORT分类为退行性、纤维化性或水肿性，以便于跨疾病比较和预后分层。在临床上，ORT作为慢性视网膜损伤的非渗出性生物标志物，有助于鉴别诊断和指导治疗策略。在年龄相关性黄斑变性中，ORT与视网膜下纤维化和视力不良有关；在地理萎缩中，它可能表明病变进展较慢。在遗传性视网膜营养不良症中，ORT反映了基因型特异性脆弱性和残留的光感受器存活，具有治疗靶向性。随着成像技术的进步，ORT有望作为疾病慢性、光感受器恢复力和勒细胞可塑性的结构标记物，提高诊断精度，并支持其作为临床试验中有意义的终点的作用。

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引用次数: 0

Structural photoreceptor evaluation in age-related macular degeneration. A comprehensive review of methods and clinical significance 年龄相关性黄斑变性的结构光感受器评价。综合综述方法及临床意义。

IF 14.7 1区医学 Q1 OPHTHALMOLOGY

Progress in Retinal and Eye Research

Pub Date : 2026-03-01 Epub Date: 2026-02-13 DOI: 10.1016/j.preteyeres.2026.101447

Lourdes Vidal-Oliver , Davide Garzone , Lukas Schloesser , Sarah Thiele , Maximilian Pfau , Wolf M. Harmening , Julius Ameln , Rosa Dolz-Marco , Nicolas Cuenca , Isabel Ortuño-Lizaran , Zhichao Wu , Robyn H. Guymer , Robert P. Finger

Age-related macular degeneration (AMD) is a disease that primarily affects the outer retina, with progressive photoreceptor degeneration and atrophy of the retinal pigment epithelium (RPE). Advances in imaging now enable photoreceptor changes to be detected and quantified with unprecedented sensitivity, whereas comparable biomarkers of RPE dysfunction remain less developed. As such, photoreceptor-based biomarkers are increasingly considered potential surrogates for current clinical trial endpoints. This review examines the current imaging modalities—particularly optical coherence tomography (OCT) and modalities enhanced by adaptive optics (AO) —used to evaluate photoreceptor structure in AMD. We explore the intrinsic value of parameters such as outer nuclear layer thickness, external limiting membrane integrity, photoreceptor inner and outer segment thickness, ellipsoid zone (EZ) integrity, and EZ reflectivity on OCT, and cone density and regularity on AO imaging, highlighting their potential and limitations. While OCT-based metrics are the most accessible in clinical settings, their clinical utility is hampered by inconsistent segmentation protocols and methodological heterogeneity. AO imaging offers unmatched resolution but faces practical barriers to widespread adoption. The field is moving in a promising direction with emerging computational tools and artificial intelligence improving accuracy and scalability. However, progress is contingent on establishing consensus definitions, standardized acquisition and analysis protocols, and normative datasets. Future efforts should focus on translating high-resolution imaging into robust, reproducible biomarkers that can be widely adopted in both clinical practice and therapeutic development.

年龄相关性黄斑变性（AMD）是一种主要影响外视网膜的疾病，伴有进行性光感受器变性和视网膜色素上皮（RPE）萎缩。成像技术的进步使光感受器的变化能够以前所未有的灵敏度检测和量化，而RPE功能障碍的可比生物标志物仍然很少开发。因此，基于光感受器的生物标志物越来越被认为是当前临床试验终点的潜在替代品。本文综述了目前用于评估AMD光感受器结构的成像方式，特别是光学相干断层扫描（OCT）和自适应光学增强的成像方式（AO）。我们探讨了外核层厚度、外限制膜完整性、光感受器内外段厚度、椭球区完整性和反射率等参数在OCT上的内在价值，以及AO成像上的锥密度和规则性等参数的内在价值，突出了它们的潜力和局限性。虽然基于oct的指标在临床环境中是最容易获得的，但它们的临床应用受到不一致的分割协议和方法异质性的阻碍。AO成像提供了无与伦比的分辨率，但面临广泛采用的实际障碍。随着新兴的计算工具和人工智能提高准确性和可扩展性，该领域正朝着一个有前途的方向发展。然而，进展取决于建立共识定义、标准化采集和分析协议以及规范数据集。未来的努力应该集中在将高分辨率成像转化为强大的，可重复的生物标志物，可以广泛应用于临床实践和治疗开发。

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引用次数: 0

Lens aging and disease: Molecular mechanisms, functional consequences, and pharmacological implications 晶状体老化和疾病：分子机制、功能后果和药理学意义

IF 14.7 1区医学 Q1 OPHTHALMOLOGY

Progress in Retinal and Eye Research

Pub Date : 2026-03-01 Epub Date: 2026-02-10 DOI: 10.1016/j.preteyeres.2026.101446

Xingjun Fan , Vincent M. Monnier

Age-related cataract (ARC) remains the leading cause of blindness worldwide, reflecting the progressive failure of lifelong mechanisms that preserve the transparency and refractive precision of the ocular lens. The lens is uniquely vulnerable to aging because its core fiber cells, crystallin proteins, and lipids persist for decades without turnover, relying on stable protein solubility, redox homeostasis, membrane integrity, and tightly coordinated epithelial renewal. With age, gradual biochemical and biomechanical shifts, including crystallin oxidation and truncation, deamidation and racemization, glycation, formation of disulfide and non-disulfide crosslinks, membrane remodeling, and stiffening of the lens nucleus, erode the structural and optical properties required for clear vision. Decline in glutathione (GSH) synthesis and redox-repair enzymes amplifies oxidative damage, while emerging evidence identifies ferroptosis, rather than apoptosis, as a dominant regulated cell-death pathway compromising lens epithelial cell survival in aging. In parallel, genome-wide association studies and exome sequencing have revealed a complex polygenic architecture for ARC, highlighting modifier genes that influence cytoskeletal resilience, protein stability, ion transport, and systemic metabolic signaling. Environmental exposures, including ultraviolet radiation, smoking, heat stress, and air pollution, interact with these genetic and biochemical pathways to accelerate opacity formation. This review integrates recent advances in lens biology, proteomics, redox regulation, lipid and membrane biophysics, mechanobiology, and cell death signaling to provide a cohesive framework for understanding how age-related changes converge to produce cataract. We also outline emerging therapeutic strategies that target redox buffering, crystallin stability, epithelial survival pathways, and biomechanical properties of the aging lens.

年龄相关性白内障（ARC）仍然是世界范围内致盲的主要原因，反映了保持晶状体透明度和屈光精度的终身机制的逐渐失效。晶状体特别容易老化，因为晶状体的核心纤维细胞、晶体蛋白和脂质会持续存在数十年而不会更新，这依赖于稳定的蛋白质溶解度、氧化还原稳态、膜完整性和紧密协调的上皮更新。随着年龄的增长，晶体蛋白氧化和截断、脱酰胺和外消旋、糖基化、二硫化物和非二硫化物交联的形成、膜重塑和晶状体核的硬化等逐渐发生的生化和生物力学变化，侵蚀了清晰视力所需的结构和光学性质。谷胱甘肽（GSH）合成和氧化还原修复酶的下降会放大氧化损伤，而新出现的证据表明，铁凋亡而不是细胞凋亡是衰老过程中影响晶状体上皮细胞存活的主要调控细胞死亡途径。与此同时，全基因组关联研究和外显子组测序揭示了ARC的复杂多基因结构，突出了影响细胞骨架弹性、蛋白质稳定性、离子转运和全身代谢信号传导的修饰基因。环境暴露，包括紫外线辐射、吸烟、热应激和空气污染，与这些遗传和生化途径相互作用，加速不透明的形成。本文综述了晶状体生物学、蛋白质组学、氧化还原调控、脂质和膜生物物理学、机械生物学和细胞死亡信号的最新进展，为理解年龄相关变化如何汇聚产生白内障提供了一个有衔接的框架。我们还概述了针对氧化还原缓冲、晶体蛋白稳定性、上皮存活途径和老化晶状体生物力学特性的新兴治疗策略。

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引用次数: 0

Ocular gene therapy mediated by AAV vectors: an overview of immunosuppressive treatments and immunomonitoring of patients involved in clinical trials AAV载体介导的眼部基因治疗：免疫抑制治疗和临床试验患者免疫监测综述

IF 14.7 1区医学 Q1 OPHTHALMOLOGY

Progress in Retinal and Eye Research

Pub Date : 2026-03-01 Epub Date: 2026-01-30 DOI: 10.1016/j.preteyeres.2026.101443

Duohao Ren , Gaelle A. Chauveau , Emilie Cabon , Julie Vendomèle , Catherine Vignal-Clermont , Hanadi Saliba , Divya Ail , Deniz Dalkara , Sylvain Fisson

Hereditary retinal diseases (RDs) are a group of diseases caused by monogenetic or multigenetic mutations in genes mostly expressed in the photoreceptors. RDs can eventually lead to severe vision impairment or blindness. Since the 1990s, ocular gene transfer mediated by adeno-associated virus (AAV)-derived vectors has been explored to treat hereditary ocular diseases via gene supplementation or gene editing, advancing gene therapy to the clinical trial stages and to one commercial product. By the end of 2024, 142 clinical trials had been initiated for different types of RDs. Immune responses remain a major concern in AAV-mediated gene therapy. Although the eye is considered as an immune-privileged organ, studies in animals and clinical evidence have demonstrated that ocular gene therapies mediated by AAV can trigger immune responses to the vector capsid and/or to the transgene genome and product. These immune responses may compromise the efficiency and safety of the therapy. In this review, we summarize clinical trials treating RDs with AAV and provide a comprehensive overview of reported immune responses, including local inflammation and systemic adaptive immune responses. Additionally, this review emphasizes that immunosuppression and immunomonitoring strategies are not yet standardized both for intraocular and systemic gene therapy clinical trials, and do not allow for accurate follow-up of side effects. It highlights the inter-individual variability among patients, reinforcing the need to evaluate multiple key immune parameters, including sensitive inflammation biomarkers, and to assess the impact of different immunosuppression regimens.

遗传性视网膜疾病（RDs）是由光感受器中表达的基因发生单基因或多基因突变引起的一组疾病。rd最终会导致严重的视力障碍或失明。自20世纪90年代以来，人们开始探索由腺相关病毒（AAV）衍生载体介导的眼部基因转移，通过基因补充或基因编辑治疗遗传性眼病，将基因治疗推进到临床试验阶段，并成为一种商业产品。截至2024年底，开展了142项不同类型rd的临床试验。在aav介导的基因治疗中，免疫反应仍然是一个主要问题。虽然眼睛被认为是一种免疫特权器官，但动物研究和临床证据表明，由AAV介导的眼部基因治疗可以触发对载体衣壳和/或转基因基因组和产物的免疫反应。这些免疫反应可能会降低治疗的效率和安全性。在这篇综述中，我们总结了用AAV治疗rd的临床试验，并全面概述了报道的免疫反应，包括局部炎症和全身适应性免疫反应。此外，本综述强调眼内和全身基因治疗临床试验的免疫抑制和免疫监测策略尚未标准化，并且无法对副作用进行准确的随访。它强调了患者之间的个体差异，加强了评估多个关键免疫参数的需求，包括敏感的炎症生物标志物，并评估不同免疫抑制方案的影响。

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引用次数: 0

IF 14.7 1区医学 Q1 OPHTHALMOLOGY

Progress in Retinal and Eye Research

Pub Date : 2026-03-01 Epub Date: 2026-02-16 DOI: 10.1016/j.preteyeres.2026.101449

Whitney Stuard Sambhariya , Catherine Bowes Rickman , Patricia A. D'Amore , Giulia Corradetti , Gregory S. Hageman , Gareth R. Howell , Olivia J. Marola , Hemali Phatnani , Nancy J. Philp , Debasish Sinha , Christopher B. Toomey , Faith Stone , Charles Eberhart , James T. Handa

Age-related macular degeneration (AMD) and Alzheimer's disease (AD) are neurodegenerative conditions that afflict millions of elderly people around the world. AMD is a progressive retinal disorder that leads to central vision loss whereas AD primarily causes cognitive decline and behavioral changes. While each disease has distinct clinical manifestations, the accumulation of extracellular amyloid-β is a common histopathologic finding. Similarly, cerebral amyloid angiopathy (CAA), a vascular condition that can exist independent or with AD, is characterized by the accumulation of amyloid-β in cerebral blood vessels. While significant investigation of the pathophysiologic links between AMD and AD has been conducted, the underlying similarities and differences in the pathobiology of AMD and CAA has not been considered. In this review, we discuss the common pathological features of these two conditions. We then discuss the similar pathobiology that involves cholesterol metabolism, apolipoprotein E, amyloid-β, and complement mediated inflammation. At the same time, we discuss key differences in their pathobiology. This discussion sheds new perspective and insights of their pathobiology.

年龄相关性黄斑变性（AMD）和阿尔茨海默病（AD）是困扰全世界数百万老年人的神经退行性疾病。AMD是一种进行性视网膜疾病，可导致中枢视力丧失，而AD主要引起认知能力下降和行为改变。虽然每种疾病都有不同的临床表现，但细胞外淀粉样蛋白-β的积累是一种常见的组织病理学发现。同样，脑淀粉样血管病（CAA）是一种可以独立存在或与AD一起存在的血管疾病，其特征是脑血管中淀粉样蛋白-β的积累。虽然已经对AMD和AD之间的病理生理联系进行了重要的研究，但AMD和CAA在病理生物学上的潜在异同尚未得到考虑。在这篇综述中，我们讨论这两种疾病的共同病理特征。然后我们讨论了类似的病理生物学，包括胆固醇代谢、载脂蛋白E、淀粉样蛋白β和补体介导的炎症。同时，我们讨论了它们在病理生物学上的关键差异。这一讨论为其病理生物学提供了新的视角和见解。

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引用次数: 0

Epigenetic reprogramming for ocular aging and disease: Mechanisms, biomarkers, and the road to the clinic 眼老化和疾病的表观遗传重编程：机制、生物标志物和临床之路

IF 14.7 1区医学 Q1 OPHTHALMOLOGY

Progress in Retinal and Eye Research

Pub Date : 2026-03-01 Epub Date: 2026-01-21 DOI: 10.1016/j.preteyeres.2026.101442

Junwon Lee , Minseok Han , Kaixiang Wang , L. Rainer Butler , David A. Sinclair

The eye's visual function relies on retinal neural cells that are long-lived, post-mitotic, and possess minimal regenerative capacity. These combined properties render them exceptionally vulnerable to the cumulative damage that drives age-related functional decline. Accumulating evidence now implicates epigenetic alterations, such as aberrant DNA methylation and histone modifications, not merely as correlates of aging but as fundamental drivers of aging and disease. These changes disrupt the stable gene expression programs required to maintain cellular identity and function, thereby contributing to the pathogenesis of irreversible blinding diseases like glaucoma and age-related macular degeneration (AMD). Unlike immutable genetic mutations, the reversible nature of these epigenetic marks offers a novel therapeutic paradigm. Epigenetic reprogramming, a strategy involving the transient expression of Yamanaka factors or chemical cocktails, provides a powerful means to reset this dysregulated epigenetic landscape and restore cells to a more youthful state. Compelling preclinical studies have validated this approach by demonstrating vision restoration in models of optic neuropathy through the rejuvenation of damaged and aged neurons. This review provides a comprehensive overview of ocular aging from an epigenetic perspective, examines the promise and potential concerns of epigenetic reprogramming, and discusses the future of rejuvenation therapies in ophthalmology.

眼睛的视觉功能依赖于视网膜神经细胞，这些细胞是长寿命的，有丝分裂后的，并且具有最小的再生能力。这些综合特性使它们特别容易受到累积损伤，从而导致与年龄相关的功能衰退。越来越多的证据表明，表观遗传改变，如异常的DNA甲基化和组蛋白修饰，不仅是衰老的相关因素，而且是衰老和疾病的基本驱动因素。这些变化破坏了维持细胞身份和功能所需的稳定基因表达程序，从而导致了青光眼和年龄相关性黄斑变性（AMD）等不可逆致盲疾病的发病机制。与不可变的基因突变不同，这些表观遗传标记的可逆性提供了一种新的治疗范式。表观遗传重编程是一种涉及山中因子或化学混合物的短暂表达的策略，它提供了一种强大的手段来重置这种失调的表观遗传景观，使细胞恢复到更年轻的状态。令人信服的临床前研究证实了这种方法，通过损伤和老化神经元的再生，在视神经病变模型中证明了视力恢复。本文从表观遗传学的角度对眼老化进行了全面的综述，探讨了表观遗传重编程的前景和潜在的问题，并讨论了眼科年轻化治疗的未来。

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引用次数: 0

Vogt-Koyanagi-Harada disease under the lens: Insights from multimodal ocular imaging 镜头下的Vogt-Koyanagi-Harada病：来自多模态眼成像的见解。

IF 14.7 1区医学 Q1 OPHTHALMOLOGY

Progress in Retinal and Eye Research

Pub Date : 2026-03-01 Epub Date: 2026-02-04 DOI: 10.1016/j.preteyeres.2026.101445

Lan Xia , Ling Chen , Lingyu Dai , Pei Zhang , Carlos Cifuentes-González , Vishali Gupta , Rupesh Agrawal , Peizeng Yang

Vogt-Koyanagi-Harada (VKH) disease is a multisystem autoimmune condition targeting melanocyte-rich ocular tissues, with inflammation primarily affecting the choroid. Despite advances in diagnostic criteria, disease staging and therapeutic decisions still rely heavily on clinical observation, with limited integration of imaging biomarkers. This review examines the evolving role of multimodal ocular imaging (MMI) in the diagnosis, monitoring, and prognostication of VKH.

We critically appraise the utility and limitations of color fundus photography, fluorescein angiography (FFA), indocyanine green angiography (ICGA), optical coherence tomography (OCT), OCT angiography (OCTA), fundus autofluorescence, B-scan ultrasonography, ultrasound biomicroscopy and anterior segment photography. These modalities provide complementary insights into disease activity, from subclinical choroiditis and retinal pigment epithelium disruption to chronic sequelae such as subretinal fibrosis and choroidal atrophy. Key imaging features—such as choroidal thickening, hypofluorescent dark dots, RPE undulations, and flow voids—are described in the context of VKH pathophysiology and disease stage.

We identify critical gaps in current practice, including the lack of standardized imaging-based definitions for relapse and remission, limited use of anterior segment imaging, and variability in access to advanced modalities. To address these challenges, we propose a stage-specific imaging framework to guide VKH assessment from prodromal to chronic phases.

MMI has transitioned from a supportive to a central role in VKH management. Future efforts should prioritize the development of quantitative imaging biomarkers, standardization of grading systems, and integration of imaging into therapeutic algorithms to support timely, personalized care and prevent irreversible visual loss.

Vogt-Koyanagi-Harada （VKH）病是一种针对富含黑素细胞的眼部组织的多系统自身免疫性疾病，炎症主要影响脉络膜。尽管诊断标准有所进步，但疾病分期和治疗决策仍然严重依赖临床观察，影像生物标志物的整合有限。本文综述了多模态眼成像（MMI）在VKH的诊断、监测和预测中的作用。我们批判性地评估彩色眼底摄影、荧光素血管造影（FFA）、吲哚菁绿血管造影（ICGA）、光学相干断层扫描（OCT）、OCT血管造影（OCTA）、眼底自体荧光、b超、超声生物显微镜和前段摄影的实用性和局限性。这些模式提供了对疾病活动的补充见解，从亚临床脉络膜炎和视网膜色素上皮破坏到慢性后遗症，如视网膜下纤维化和脉络膜萎缩。关键的影像学特征，如脉络膜增厚、低荧光黑点、RPE波动和流动空洞，在VKH病理生理和疾病分期的背景下被描述。我们确定了当前实践中的关键差距，包括缺乏基于复发和缓解的标准化成像定义，前节段成像的使用有限，以及获得高级模式的可变性。为了应对这些挑战，我们提出了一个特定阶段的成像框架，以指导从前驱期到慢性期的VKH评估。MMI已经从VKH管理的支持角色转变为核心角色。未来的努力应优先发展定量成像生物标志物，标准化分级系统，并将成像整合到治疗算法中，以支持及时，个性化的护理和防止不可逆转的视力丧失。

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引用次数: 0