Department of Ophthalmology, University of Bonn, Bonn, Germany.
Department of Ophthalmology and Westmead Institute for Medical Research, University of Sydney, Sydney, Australia.
Ophthalmology. 2018 Mar;125(3):369-390. doi: 10.1016/j.ophtha.2017.08.038. Epub 2017 Oct 27.
Geographic atrophy (GA) is an advanced form of age-related macular degeneration (AMD) that leads to progressive and irreversible loss of visual function. Geographic atrophy is defined by the presence of sharply demarcated atrophic lesions of the outer retina, resulting from loss of photoreceptors, retinal pigment epithelium (RPE), and underlying choriocapillaris. These lesions typically appear first in the perifoveal macula, initially sparing the foveal center, and over time often expand and coalesce to include the fovea. Although the kinetics of GA progression are highly variable among individual patients, a growing body of evidence suggests that specific characteristics may be important in predicting disease progression and outcomes. This review synthesizes current understanding of GA progression in AMD and the factors known or postulated to be relevant to GA lesion enlargement, including both affected and fellow eye characteristics. In addition, the roles of genetic, environmental, and demographic factors in GA lesion enlargement are discussed. Overall, GA progression rates reported in the literature for total study populations range from 0.53 to 2.6 mm/year (median, ∼1.78 mm/year), assessed primarily by color fundus photography or fundus autofluorescence (FAF) imaging. Several factors that could inform an individual's disease prognosis have been replicated in multiple cohorts: baseline lesion size, lesion location, multifocality, FAF patterns, and fellow eye status. Because best-corrected visual acuity does not correspond directly to GA lesion enlargement due to possible foveal sparing, alternative assessments are being explored to capture the relationship between anatomic progression and visual function decline, including microperimetry, low-luminance visual acuity, reading speed assessments, and patient-reported outcomes. Understanding GA progression and its individual variability is critical in the design of clinical studies, in the interpretation and application of clinical trial results, and for counseling patients on how disease progression may affect their individual prognosis.
地图状萎缩(GA)是一种与年龄相关的黄斑变性(AMD)的晚期形式,导致视觉功能进行性和不可逆转的丧失。地图状萎缩是由外视网膜的明显边界萎缩病变定义的,这些病变是由光感受器、视网膜色素上皮(RPE)和下面的脉络膜毛细血管丧失引起的。这些病变通常首先出现在周边黄斑,最初避开中心凹,随着时间的推移,通常会扩大并融合包括中心凹。尽管个体患者之间 GA 进展的动力学高度可变,但越来越多的证据表明,某些特征可能对预测疾病进展和结局很重要。这篇综述综合了目前对 AMD 中 GA 进展的理解,以及已知或推测与 GA 病变扩大相关的因素,包括受影响眼和对侧眼的特征。此外,还讨论了遗传、环境和人口统计学因素在 GA 病变扩大中的作用。总的来说,文献中报告的总研究人群中 GA 进展的速度范围为 0.53 至 2.6 毫米/年(中位数约为 1.78 毫米/年),主要通过眼底彩色照相或眼底自发荧光(FAF)成像评估。多个因素在多个队列中得到了复制,可以为个体的疾病预后提供信息:基线病变大小、病变位置、多灶性、FAF 模式和对侧眼状态。由于最佳矫正视力由于可能存在中心凹保留而不能直接对应于 GA 病变扩大,因此正在探索替代评估方法来捕捉解剖进展与视觉功能下降之间的关系,包括微视野计、低亮度视力、阅读速度评估和患者报告的结果。了解 GA 进展及其个体变异性对于临床研究的设计、临床试验结果的解释和应用以及为患者提供关于疾病进展如何影响其个体预后的信息都至关重要。