Peripheral Retinal Vascular Biomarkers on Ultra-Widefield OCT Angiography for Severity Grading in Retinitis Pigmentosa.

Authors

Yutian Jiao, Jingwen Jiang, Zhixuan Chen, Bing Lin, Hong Wang, Chenyue Hang, Ting Zhang, Xiaoling Wan, Jianqing Li, Tianyuan Zhao, Hao Zhou, Xujun Jiang, Weiqiao Xia, Tong Li, Junran Sun, Xinxin Liu, Xiangjun She, Yun Zhang, Huixun Jia, Yuanyuan Gong, Jingming Li, Suqin Yu, Jieqiong Chen, Xiaodong Sun

Published in

American journal of ophthalmology. Aug 23, 2026. Epub Aug 23, 2026.

Abstract

To identify peripheral retinal vascular phenotypes in retinitis pigmentosa (RP) using ultra-widefield OCT angiography (UWF-OCTA), validate a quantitative vascular biomarker, and develop a clinically applicable severity grading system.
Development and validation of a severity grading system.
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 threshold (FST), and static perimetry (SP)-were evaluated using linear mixed-effects models (LMMs) to account for inter-eye correlation. A four-stage severity grading system was developed using principal component analysis (PCA) to construct a composite functional score and regression tree analysis with cluster bootstrapping (1,000 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.
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 (BCVA) in both internal (η² = 0.17) and external (η² = 0.28) validation cohorts.
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.

PMID:
42633834
Bibliographic data and abstract were imported from PubMed on 24 Aug 2026.

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