Jacobs Retina Center, University of California San Diego, La Jolla, California.
Princess Alexandra Eye Pavilion, University of Edinburgh, Edinburgh, United Kingdom.
Retina. 2021 Jun 1;41(6):1329-1337. doi: 10.1097/IAE.0000000000003017.
To identify quantifiable markers of disease progression in patients with foveal-sparing atrophic late-onset retinal degeneration using fundus autofluorescence and spectral-domain optical coherence tomography imaging.
Natural history study evaluating patients within a 3-year interval. Disease progression was assessed based on the area of retinal atrophy, macular topographic distribution of lesions, retinal and choroidal thickness and volume, and choroidal vascularity index.
Twenty-four eyes (12 individuals) were included for fundus autofluorescence, and 31 eyes (16 individuals) for spectral-domain optical coherence tomography studies. Measurements were symmetrical between eyes of the same patient. The area of atrophy significantly enlarged (P = 0.002), with a growth rate of 2.67 mm2/year (SD: 2.13; square rooted: 0.57 mm/year, SD = 0.34). Baseline area of atrophy and progression both correlated with age. Most atrophic lesions were found in the temporal macula and progressed nasally at follow-up. Central choroidal and retinal thicknesses and volume in late-onset retinal degeneration cases were significantly reduced compared with controls, but only central retinal thickness decreased significantly at follow-up.
This study identifies the area of atrophy and central retinal thickness, but not chorioretinal volume or choroidal thickness, as markers of short-term progression in late-onset retinal degeneration. These findings may be useful for disease monitoring and late-onset retinal degeneration interventional studies.
利用眼底自发荧光和频域光相干断层扫描成像，确定黄斑区 spared 的萎缩性晚发性视网膜变性患者疾病进展的量化标志物。
对 3 年内的患者进行自然史研究。根据视网膜萎缩面积、黄斑病变的眼底地形图分布、视网膜和脉络膜厚度及体积、脉络膜血流指数评估疾病进展。
共纳入 24 只眼（12 例）进行眼底自发荧光检查，31 只眼（16 例）进行频域光相干断层扫描检查。同一患者双眼的测量值是对称的。萎缩面积显著增大（P = 0.002），增长率为 2.67 mm2/年（标准差：2.13；平方根：0.57 mm/年，标准差 = 0.34）。基线萎缩面积和进展均与年龄相关。大多数萎缩性病变位于颞侧黄斑，随访时向鼻侧进展。与对照组相比，晚发性视网膜变性患者的中心脉络膜和视网膜厚度及体积显著降低，但仅中心视网膜厚度在随访时显著降低。
本研究确定了萎缩面积和中心视网膜厚度，而不是脉络膜视网膜体积或脉络膜厚度，作为晚发性视网膜变性短期进展的标志物。这些发现可能有助于疾病监测和晚发性视网膜变性干预研究。
