玻璃膜疣和厚玻璃膜疣:定义、发病机制和临床意义。
Drusen and pachydrusen: the definition, pathogenesis, and clinical significance.
机构信息
Beijing Institute of Ophthalmology, Beijing Tongren Eye Center, Beijing Tongren Hospital, Capital Medical University, Beijing, 100730, PR China.
NIHR Moorfields Biomedical Research Centre, Moorfields Eye Hospital, London, UK.
出版信息
Eye (Lond). 2021 Jan;35(1):121-133. doi: 10.1038/s41433-020-01265-4. Epub 2020 Nov 18.
The pachychoroid disease spectrum encompasses seven major retinal conditions including central serous chorioretinopathy (CSC), polypoidal choroidal vasculopathy (PCV), and pachychoroid neovasculopathy or type I macular neovascularisation (MNV) secondary to chronic persistent thickening and dysfunction of the choroidal vasculature. Drusen are focal yellow-white deposits of extracellular debris, which consist of complement proteins, esterified and nonesterified cholesterol, apolipoproteins, carbohydrates, and trace elements, above the retinal pigment epithelium (RPE) or between the RPE and Bruch's membrane. Although drusen are an essential disease precursor of advanced age-related macular degeneration (AMD), a new entity "pachydrusen" has been identified to be associated with some of the enitites that constitute the pachychoroid spectrum. It remains to be determined what the exact differences are between soft drusen, pseudodrusen, and pachydrusen in terms of phenotype, genotype, and pathogenesis. Improving our knowledge in these areas will inevitably improve our understanding of their clinical significance especially as in disease prediction in AMD and the pachychroid spectrum disorders. It remains controversial whether PCV is a subtype of AMD. Understanding the pathogenesis of different types of drusen may also help in addressing if phenotype and/or genotype of type 1 MNV associated with pachychoroid are similar to type 1 MNV related to AMD. Furthermore, because pachydrusen links two pachychoroid diseases, CSC and PCV, it is also of great interest to investigate if CSC is an early stage or a predictor of PCV in future research. In this review, we share our experience in clinical practice and the latest published evidence-based literature to emphasize the differences and similarities in morphology, pathogenesis, and clinical significance of drusen and pachydrusen, a new member of the pachychoroid spectrum disorders.
脉络膜增厚疾病谱包括七种主要的视网膜疾病,包括中心性浆液性脉络膜视网膜病变(CSC)、息肉样脉络膜血管病变(PCV)和脉络膜新生血管病变或 I 型黄斑新生血管化(MNV),这些疾病继发于脉络膜血管的慢性持续性增厚和功能障碍。脉络膜增厚疾病谱包括七种主要的视网膜疾病,包括中心性浆液性脉络膜视网膜病变(CSC)、息肉样脉络膜血管病变(PCV)和脉络膜新生血管病变或 I 型黄斑新生血管化(MNV),这些疾病继发于脉络膜血管的慢性持续性增厚和功能障碍。Drusen 是视网膜色素上皮(RPE)上方或 RPE 与 Bruch 膜之间的细胞外碎片的局灶性黄白色沉积,由补体蛋白、酯化和非酯化胆固醇、载脂蛋白、碳水化合物和微量元素组成。Drusen 是与年龄相关的黄斑变性(AMD)相关的高级别疾病的重要疾病前体,但已确定一种新实体“厚 drusen”与构成脉络膜增厚谱的一些实体有关。目前尚不清楚在表型、基因型和发病机制方面,软 drusen、假性 drusen 和厚 drusen 之间的确切差异是什么。提高我们在这些领域的知识将不可避免地提高我们对它们的临床意义的理解,特别是在 AMD 和脉络膜增厚谱疾病的疾病预测方面。PCV 是否为 AMD 的亚型仍存在争议。了解不同类型 drusen 的发病机制也有助于解决与脉络膜增厚相关的 I 型 MNV 的表型和/或基因型是否与 AMD 相关的 I 型 MNV 相似。此外,由于厚 drusen 将两种脉络膜增厚疾病(CSC 和 PCV)联系在一起,因此研究 CSC 是否为 PCV 的早期阶段或未来研究中的预测因子也很有意义。在这篇综述中,我们分享了我们在临床实践中的经验和最新发表的循证文献,强调了 drusen 和厚 drusen 在形态、发病机制和临床意义上的差异和相似之处,厚 drusen 是脉络膜增厚谱疾病的新成员。
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