短波和近红外自发荧光测量的隐性斯塔加特病的视网膜色素上皮萎缩。

Retinal Pigment Epithelium Atrophy in Recessive Stargardt Disease as Measured by Short-Wavelength and Near-Infrared Autofluorescence.

机构信息

Department of Ophthalmology, Edward S. Harkness Eye Institute, Columbia University Medical Center, New York, NY, USA.

Jonas Children's Vision Care, New York, NY, USA.

出版信息

Transl Vis Sci Technol. 2021 Jan 5;10(1):3. doi: 10.1167/tvst.10.1.3. eCollection 2021 Jan.

PURPOSE

To compare the detection of retinal pigment epithelium (RPE) atrophy in short-wavelength (SW-AF) and near-infrared autofluorescence (NIR-AF) images in Stargardt disease (STGD1) patients.

METHODS

SW-AF and NIR-AF images (115 eyes from 115 patients) were analyzed by two independent graders. Hypoautofluorescent (hypoAF) areas, indicative of RPE atrophy, were measured, and the two modalities were compared.

RESULTS

Patients were segregated into four groups: nascent (6 [5%]), widespread (21 [18%]), discrete (55 [48%]), and chorioretinal atrophy (33 [29%]). The areas of hypoAF were larger in NIR-AF compared to SW-AF images in discrete (3.9 vs. 2.2 mm, < 0.001) and chorioretinal atrophy (12.7 vs. 11.4 mm, = 0.015). Similar findings were observed qualitatively in nascent and widespread atrophy patients. Using the area linear model (ALM), lesion area increased at similar rates in SW-AF and NIR-AF images of discrete atrophy (0.20 vs. 0.32 mm/y, = 0.275) and chorioretinal atrophy (1.30 vs. 1.74 mm/y, = 0.671). Using the radius linear model (RLM), the lesion effective radius also increased similarly in SW-AF and NIR-AF images in discrete (0.03 vs. 0.05 mm/y, = 0.221) and chorioretinal atrophy (0.08 vs. 0.10 mm/y, = 0.754) patients.

CONCLUSIONS

NIR-AF reveals a larger area of RPE atrophy in STGD1 patients compared to SW-AF images, but rates of lesion enlargement in the two modalities are similar.

TRANSLATIONAL RELEVANCE

Measurements of RPE atrophy by AF imaging are crucial for monitoring STGD1 disease progression and given our findings we advocate greater use of NIR-AF for patients.

目的

比较短波（SW-AF）和近红外自发荧光（NIR-AF）图像在斯塔加特病（STGD1）患者中检测视网膜色素上皮（RPE）萎缩的效果。

方法

由两位独立的评分员分析 SW-AF 和 NIR-AF 图像（来自 115 名患者的 115 只眼）。测量指示 RPE 萎缩的低自发荧光（hypoAF）区域，并比较两种模式。

结果

患者分为四组：初发（6 例[5%]）、广泛（21 例[18%]）、离散（55 例[48%]）和脉络膜视网膜萎缩（33 例[29%]）。与 SW-AF 图像相比，在离散型（3.9 毫米对 2.2 毫米，＜0.001）和脉络膜视网膜萎缩型（12.7 毫米对 11.4 毫米， = 0.015）中，NIR-AF 图像中的 hypoAF 区域更大。在初发和广泛萎缩患者中，定性观察也有类似发现。使用面积线性模型（ALM），SW-AF 和 NIR-AF 图像中离散性萎缩（0.20 毫米对 0.32 毫米/年， = 0.275）和脉络膜视网膜萎缩（1.30 毫米对 1.74 毫米/年， = 0.671）的病变面积以相似的速率增加。使用半径线性模型（RLM），在离散型（0.03 毫米对 0.05 毫米/年， = 0.221）和脉络膜视网膜萎缩（0.08 毫米对 0.10 毫米/年， = 0.754）患者中，SW-AF 和 NIR-AF 图像中的病变有效半径也以相似的方式增加。