USH2A相关视网膜变性（RUSH2A）研究中4年视野丧失进展速率的特征分析。
Characterization of Visual Field Loss Over 4 Years in the Rate of Progression in USH2A-Related Retinal Degeneration (RUSH2A) Study.
作者信息
Duncan Jacque L, Maguire Maureen G, McDaniel Lee S, Doucet Nicole R, Audo Isabelle, Ayala Allison R, Cheetham Janet K, Cheng Peiyao, Durham Todd A, Huckfeldt Rachel M, Hufnagel Robert B, Jayasundera K Thiran, Khan Naheed, Malbin Brett, Maldonado Ramiro S, Michaelides Michel, Pennesi Mark E, Weng Christina Y, Zmejkoski Alex, Aravind Shobana, Ishikawa Hiroshi, Birch David G
机构信息
From the University of California (J.L.D.), San Francisco, California, USA.
Jaeb Center for Health Research (M.G.M., L.S.M., N.R.D., A.R.A., and P.C.), Tampa, Florida, USA.
出版信息
Am J Ophthalmol. 2025 Mar 27;276:9-21. doi: 10.1016/j.ajo.2025.03.039.
PURPOSE
To report visual field loss using static perimetry (SP) and kinetic perimetry (KP) over 4 years in the Rate of Progression of USH2A-related Retinal Degeneration (RUSH2A) study.
DESIGN
Prospective, observational cohort study.
SUBJECTS, PARTICIPANTS, AND/OR CONTROLS: Participants had USH2A-related rod-cone degeneration, visual acuity ≥20/80, and KP III4e ≥10° at baseline in the study eye. Preserved cohorts with baseline visual fields sufficient to detect progression were identified.
METHODS
Participants were examined annually through 4 years. Mixed-effects models were used to estimate the annual, standardized rate, and percentage rates of change.
MAIN OUTCOME MEASURES
SP measures included hill of vision (total: V, central 30°: V, and peripheral: V) and centrally weighted mean sensitivity (MScw). Percentages with 4-year progression exceeding the coefficient of repeatability (CoR) and with change meeting Food and Drug Administration (FDA)-recommended criteria were estimated. KP seeing area (dB-steradian (sr)/degree) for I4e, III4e, and V4e isopters was calculated.
RESULTS
The average decline with SP (95% CI) was 1.94 (1.62, 2.25) dB-sr/y for V, 0.54 (0.45, 0.62) dB-sr/y for V, 1.37 (1.11, 1.63) dB-sr/y for V and 0.56 (0.48, 0.64) dB/y for MS. Average percentage decline per year was 8.6% (7.2, 10.0) for V, 6.4% (5.3, 7.5) for V, 13.6% (10.4, 16.7) for V, and 5.6% (4.7, 6.4) for MS. The standardized rate of change was greatest at -1.35 for MS. Rates were higher in the preserved cohorts. Progression exceeding the CoR was 18% (11, 28) for V, 21% (13, 31) for V, 21% (13, 31) for V and 17% (10, 27) for MS. Progression exceeding an FDA-recommended threshold was 5% (2%, 12%) for all SP points and 45% (35%, 55%) for functional transition points. Average KP annual percentage decline was 13.1% (7.5, 18.5) for I4e, 12.1% (8.1,15.9) for III4e, and 9.2% (6.3,12.0) for V4e.
CONCLUSIONS
All quantitative perimetry measures declined over 4 years. Progression was greater than the CoR in a relatively low percentage of eyes (17%-21%); 45% exceeded the FDA-recommended threshold when only functional transition points were considered. Standardized rate of change was greatest for MS. These measures are useful characterizations of vision loss in USH2A-related retinal degeneration.
目的
报告在USH2A相关视网膜变性进展速率（RUSH2A）研究中，4年期间使用静态视野计（SP）和动态视野计（KP）检测到的视野丧失情况。
设计
前瞻性观察性队列研究。
受试者、参与者和/或对照：研究眼基线时，参与者患有USH2A相关的视杆 - 视锥细胞变性，视力≥20/80，且KP III4e≥10°。确定了具有足以检测进展的基线视野的保留队列。
方法
参与者每年接受检查，为期4年。使用混合效应模型估计年度标准化速率和变化百分比。
主要观察指标
SP测量包括视力峰值（总计：V、中央30°：V和周边：V）以及中央加权平均敏感度（MScw）。估计4年进展超过重复性系数（CoR）以及变化符合美国食品药品监督管理局（FDA）推荐标准的百分比。计算I4e、III4e和V4e等视线的KP可见面积（分贝 - 立体弧度（sr）/度）。
结果
SP测量的平均下降（9５％CI）为：V总计每年1.94（1.62, 2.25）dB - sr，中央30°的V每年0.54（0.45, 0.62）dB - sr，周边的V每年1.37（1.11, 1.63）dB - sr，MScw每年为0.56（0.48, 0.64）dB。每年平均下降百分比为：V总计8.6％（7.2, 10.0），中央30°的V为6.4％（5.3, 7.5），周边的V为13.6％（10.4, 16.7），MScw为5.6％（4.7, 6.4）。MScw的标准化变化率最大，为 - 1.35。保留队列中的变化率更高。进展超过CoR的比例为：V总计18％（11, 28），中央30°的V为21％（13, 31），周边的V为21％（13, 31），MScw为17％（10, 27）。超过FDA推荐阈值的进展在所有SP点为5％（2％, 12％），在功能转换点为45％（35％, 55％）。KP每年平均下降百分比为：I4e为13.1％（7.5, 18.5），III4e为12.1％（8.1, 15.9），V4e为9.2％（6.3, 12.0）。
结论
所有定量视野测量指标在4年期间均下降。进展超过CoR的眼睛比例相对较低（17％ - 21％）；仅考虑功能转换点时，45％超过了FDA推荐的阈值。MScw的标准化变化率最大。这些测量指标有助于描述USH2A相关视网膜变性中的视力丧失情况。
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