Wilmer Eye Institute, Johns Hopkins Hospital, Baltimore, Maryland, United States.
Department of Ophthalmology, Bascom Palmer Eye Institute, University of Miami Miller School of Medicine, Miami, Florida, United States.
Invest Ophthalmol Vis Sci. 2023 Mar 1;64(3):2. doi: 10.1167/iovs.64.3.2.
Age-related macular degeneration (AMD) is a leading cause of blindness among the elderly worldwide. Clinical imaging and histopathologic studies are crucial to understanding disease pathology. This study combined clinical observations of three brothers with geographic atrophy (GA), followed for 20 years, with histopathologic analysis.
For two of the three brothers, clinical images were taken in 2016, 2 years prior to death. Immunohistochemistry, on both flat-mounts and cross sections, histology, and transmission electron microscopy were used to compare the choroid and retina in GA eyes to those of age-matched controls.
Ulex europaeus agglutinin (UEA) lectin staining of the choroid demonstrated a significant reduction in the percent vascular area and vessel diameter. In one donor, histopathologic analysis demonstrated two separate areas with choroidal neovascularization (CNV). Reevaluation of swept-source optical coherence tomography angiography (SS-OCTA) images revealed CNV in two of the brothers. UEA lectin also revealed a significant reduction in retinal vasculature in the atrophic area. A subretinal glial membrane, composed of processes positive for glial fibrillary acidic protein and/or vimentin, occupied areas identical to those of retinal pigment epithelium (RPE) and choroidal atrophy in all three AMD donors. SS-OCTA also demonstrated presumed calcific drusen in the two donors imaged in 2016. Immunohistochemical analysis and alizarin red S staining verified calcium within drusen, which was ensheathed by glial processes.
This study demonstrates the importance of clinicohistopathologic correlation studies. It emphasizes the need to better understand how the symbiotic relationship between choriocapillaris and RPE, glial response, and calcified drusen impact GA progression.
年龄相关性黄斑变性（AMD）是全球老年人致盲的主要原因。临床成像和组织病理学研究对于了解疾病病理学至关重要。本研究结合了三名患有地图状萎缩（GA）的兄弟的临床观察结果，这些兄弟经过 20 年的随访，并结合了组织病理学分析。
对于其中的两名兄弟，在他们去世前两年的 2016 年拍摄了临床图像。使用免疫组织化学、平展和切片组织学以及透射电子显微镜，比较 GA 眼的脉络膜和视网膜与年龄匹配的对照组。
Ulex europaeus agglutinin（UEA）凝集素对脉络膜的染色显示血管面积和血管直径的百分比显著减少。在一名供体中，组织病理学分析显示存在两个独立的脉络膜新生血管化（CNV）区域。重新评估扫频源光学相干断层扫描血管造影（SS-OCTA）图像显示，两名兄弟中有两名存在 CNV。UEA 凝集素也显示出萎缩区域视网膜血管的显著减少。由胶质纤维酸性蛋白和/或波形蛋白阳性的过程组成的视网膜下胶质膜，占据了与所有三名 AMD 供体的 RPE 和脉络膜萎缩相同的区域。SS-OCTA 还显示了在 2016 年成像的两名供体中存在推测的钙化玻璃膜疣。免疫组织化学分析和茜素红 S 染色证实了玻璃膜疣内的钙，钙被胶质过程包裹。
本研究证明了临床组织病理学相关性研究的重要性。它强调了需要更好地了解脉络膜毛细血管和 RPE 之间的共生关系、胶质反应以及钙化玻璃膜疣如何影响 GA 的进展。
