Khan Muhammad Jehanzeb, Rustam Zainab, Aamir Faiqa Binte, Miranda Maria Chairez, Shaikh Imad, Akhlaq Anam, Liu Jiawen, Singh Mandeep, Kong Xiangrong, Campochiaro Peter A
The Wilmer Eye Institute, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA,
The Wilmer Eye Institute, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA.
Ophthalmic Res. 2025;68(1):156-168. doi: 10.1159/000543082. Epub 2025 Jan 31.
The aim of this study was to characterized fundus autofluorescence (AF) changes that occur in the macula of patients with retinitis pigmentosa (RP).
We conducted a case series on 99 patients with RP. Features seen on fundus AF images were evaluated, organized into a grading scheme, and correlated with ellipsoid zone (EZ) width. Patterns of AF changes occurring in the macula and correlation with EZ width.
Four primary fundus AF phenotypes were identified: (1) hyperAF arc, (2) hyperAF ring, (3) hyperAF ring with abnormal hyperAF within the ring, and (4) central hyperAF. The second phenotype was most common and had 3 subgroups, hyperAF rings within the macula, those extending outside the macula, and incomplete rings. HyperAF rings were also characterized as narrow or wide with wide rings having a greater amount of hyperAF. Linear Mixed-Effects Model showed mean measured EZ width was significantly greater for phenotype 1 versus each of the other 3 phenotypes (p < 0.01) and for phenotype 2 versus phenotypes 3 and 4 (p < 0.05), and also differed among phenotype 2 subgroups (p < 0.05). Other AF characteristics identified were focal posterior distinct or indistinct hypoAF which sometimes formed complete or incomplete hypoAF rings surrounding a hyperAF ring, diffuse or focal hyperAF outside hyperAF rings, and the amount of encroachment of peripheral hypoAF.
A grading scheme for macular AF features in patients with RP identified phenotypes that correlate with stage of disease based upon EZ width. Longitudinal studies are needed to test whether presumed early AF phenotypes evolve into later phenotypes. Use of the grading scheme for patient populations in interventional trials could help determine if any of the defined AF features provide predictive value for therapeutic responses.
本研究的目的是描述视网膜色素变性（RP）患者黄斑区发生的眼底自发荧光（AF）变化。
我们对99例RP患者进行了病例系列研究。评估眼底AF图像上所见的特征，将其整理成一个分级方案，并与椭圆体带（EZ）宽度相关联。黄斑区出现的AF变化模式及其与EZ宽度的相关性。
确定了四种主要的眼底AF表型：（1）高自发荧光弧，（2）高自发荧光环，（3）环内有异常高自发荧光的高自发荧光环，以及（4）中央高自发荧光。第二种表型最常见，有3个亚组，即黄斑区内的高自发荧光环、延伸至黄斑区外的高自发荧光环和不完整的环。高自发荧光环也分为窄环或宽环，宽环的高自发荧光量更大。线性混合效应模型显示，与其他3种表型相比，表型1的平均测量EZ宽度显著更大（p<0.01），与表型3和4相比，表型2的平均测量EZ宽度显著更大（p<0.05），并且表型2的亚组之间也存在差异（p<0.05）。其他确定的AF特征包括局灶性后部明显或不明显的低自发荧光，其有时形成围绕高自发荧光环的完整或不完整的低自发荧光环、高自发荧光环外的弥漫性或局灶性高自发荧光，以及周边低自发荧光的侵蚀量。
RP患者黄斑区AF特征的分级方案确定了与基于EZ宽度的疾病阶段相关的表型。需要进行纵向研究来检验假定的早期AF表型是否会演变为后期表型。在干预试验中对患者群体使用该分级方案有助于确定任何已定义的AF特征是否为治疗反应提供预测价值。
