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Peripheral Retinal Vascular Biomarkers on Ultrawidefield Optical Coherence Tomography Angiography for Severity
Yutian Jiao1, Jingwen Jiang1, Zhixuan Chen1
1From the Department of Ophthalmology (Y.J., J.J., Z.C., H.W., C.H., T.Z., X.W., J.L., T.Z., H.Z., X.J., W.X., T.L., J.S., X.L., H.J., Y.G., S.Y., J.C., X.S.), Shanghai General Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai, China; National Clinical Research Center for Eye Diseases (Y.J., J.J., Z.C., H.W., C.H., T.Z., X.W., J.L., T.Z., H.Z., X.J., W.X., T.L., J.S., X.L., H.J., Y.G., S.Y., J.C., X.S.), Shanghai, China.
Purpose:
To identify peripheral retinal vascular phenotypes in retinitis pigmentosa (RP) using ultrawidefield optical coherence tomography angiography (UWF-OCTA), validate a quantitative vascular biomarker, and develop a clinically applicable severity grading system.
Design:
Development and validation of a severity grading system.
Methods:
We analyzed 544 eyes from 272 patients with primary RP from three tertiary centers. Peripheral vascular phenotypes were defined using UWF-OCTA. Structure-function correlations between the CNZ area and multimodal visual functions-including kinetic perimetry (KP), full-field stimulus testing (FST), and static perimetry-were evaluated using linear mixed effects models to account for intereye correlation. A four-stage severity grading system was developed using principal component analysis to construct a composite functional score and regression tree analysis with cluster bootstrapping (1000 resamples) to determine optimal CNZ cut-points; the system was then validated in internal and external datasets. Age-related severity distribution was evaluated through a Kaplan-Meier age-at-observation simulation.
Results:
Two distinct peripheral vascular phenotypes were identified: Type 1 (terminal remodeling; 306 eyes, 56.2%) and Type 2 (simple atrophy; 238 eyes, 43.8%). Type 1 eyes showed greater disease severity than Type 2 eyes at comparable ages. The CNZ area demonstrated strong structure-function correlations, explaining substantial variance in KP (Marginal R2 up to 0.77) and FST (Marginal R2 up to 0.64). A CNZ-based 4-stage severity grading (Stage I, ≥260 mm²; Stage II, 200-260 mm²; Stage III, 150-200 mm²; Stage IV, <150 mm²) effectively stratified disease severity, demonstrating high explanatory power for visual function (KP η² up to 0.75; FST η² up to 0.66) and maintaining discriminatory performance for best-corrected visual acuity in both internal (η² = 0.17) and external (η² = 0.28) validation cohorts.
Conclusions:
Peripheral retinal vascular alterations captured by UWF-OCTA define distinct RP phenotypes and identify the CNZ as a robust quantitative biomarker of disease severity. The CNZ-based severity grading system provides an objective framework for clinical severity assessment, disease monitoring, and patient stratification in future interventional trials.
