Bassil Fabiana L, Colijn Johanna M, Thiadens Alberta A H J, Biarnés Marc
Department of Ophthalmology, Erasmus Medical Center (F.L.B., J.M.C., A.A.H.J.T.), Rotterdam, the Netherlands.
Oftalmologia Mèdica i Quirúrgica (OMIQ) Research (M.B.), Barcelona, Spain.
Am J Ophthalmol. 2025 Jan;269:30-48. doi: 10.1016/j.ajo.2024.07.035. Epub 2024 Aug 15.
To compare the macular retinal pigment epithelium (RPE) atrophy progression rate of selected degenerative and macular inherited retinal diseases (IRD).
Systematic review and meta-analysis.
The protocol was registered on the PROSPERO database. Medline, Embase, Web of Science, Cochrane Central Register of Controlled Trials, and Google Scholar were searched up to September 15, 2023 for articles reporting the RPE atrophy growth rate in treatment-naïve eyes with geographic atrophy (GA), Stargardt disease (STGD1), Best disease, pseudoxanthoma elasticum (PXE), central areolar choroidal dystrophy (CACD), or pattern dystrophies with no previous or current macular neovascularization and a minimum follow-up time of 12 months. Meta-analyses determined mean RPE atrophy growth rates per disease, imaging modality (fundus autofluorescence [FAF], optical coherence tomography [OCT], or color fundus photography [CFP]) and metric (mm/y or mm/y). The Newcastle-Ottawa scale and the Cochrane Risk-of-Bias tool assessed the risk of bias, and funnel plots were used to evaluate small-study effects.
From 4354 publications, 85 were included for meta-analysis: 69 studies (7815 eyes) on GA, 15 (1367 eyes) on STGD1, and one on both. Two studies on PXE were only eligible for review. No studies for other diseases met our eligibility criteria. The overall mean RPE atrophy growth rate for GA using FAF was 1.65 mm/y (95% confidence interval [CI], 1.49-1.81) and 0.35 mm/y (95% CI, 0.28-0.41); using OCT, it was 1.46 mm/y (95% CI, 1.28-1.64) and 0.34 mm/y (95% CI, 0.28-0.40); and on CFP it was 1.76 mm/y (95% CI, 1.56-1.97) and 0.30 mm/y (95% CI, 0.28-0.31). For STGD1, using FAF it was 1.0 mm/y (95% CI, 0.77-1.23) and 0.20 mm/y (95% CI, 0.17-0.23); on OCT, it was 0.80 mm/y (95% CI, 0.72-0.88). No studies on STGD1 reported the growth rate with other imaging modalities or metrics. Growth rates in GA were faster than in STGD1 (p < .05). A larger baseline area of atrophy was generally associated with faster growth rates.
The RPE atrophy growth rate in GA is faster than in STGD1 but with great variation between studies and imaging modalities. Limited information was available for other macular IRD, suggesting further research is needed.
比较特定退行性和黄斑遗传性视网膜疾病（IRD）的黄斑视网膜色素上皮（RPE）萎缩进展速率。
系统评价和荟萃分析。
该方案已在PROSPERO数据库注册。截至2023年9月15日，检索了Medline、Embase、Web of Science、Cochrane对照试验中央注册库和谷歌学术，以查找报告初治眼中地理性萎缩（GA）、Stargardt病（STGD1）、Best病、弹性假黄瘤（PXE）、中央性晕轮状脉络膜营养不良（CACD）或无既往或当前黄斑新生血管形成且随访时间至少12个月的图案性营养不良的RPE萎缩增长率的文章。荟萃分析确定了每种疾病、成像方式（眼底自发荧光[FAF]、光学相干断层扫描[OCT]或彩色眼底照相[CFP]）和指标（mm/y或mm/y）的平均RPE萎缩增长率。使用纽卡斯尔-渥太华量表和Cochrane偏倚风险工具评估偏倚风险，并使用漏斗图评估小研究效应。
从4354篇出版物中，纳入85篇进行荟萃分析：69项研究（7815只眼）关于GA，15项（1367只眼）关于STGD1，1项同时涉及两者。两项关于PXE的研究仅符合综述条件。没有其他疾病的研究符合我们的纳入标准。使用FAF时，GA的总体平均RPE萎缩增长率为1.65 mm/y（95%置信区间[CI]，1.49 - 1.81）和0.35 mm/y（95% CI，0.28 - 0.41）；使用OCT时，分别为1.46 mm/y（95% CI，1.28 - 1.64）和0.34 mm/y（95% CI，0.28 - 0.40）；使用CFP时，分别为1.76 mm/y（95% CI，1.56 - 1.97）和0.30 mm/y（95% CI，0.28 - 0.31）。对于STGD1，使用FAF时为1.0 mm/y（95% CI，0.77 - 1.23）和0.20 mm/y（95% CI，0.17 - 0.23）；使用OCT时为0.80 mm/y（95% CI，0.72 - 0.88）。没有关于STGD1的研究报告其他成像方式或指标的增长率。GA中的增长率比STGD1更快（p < 0.05）。较大的基线萎缩面积通常与更快的增长率相关。
GA中的RPE萎缩增长率比STGD1更快，但研究和成像方式之间存在很大差异。关于其他黄斑IRD的信息有限，表明需要进一步研究。
