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Decline of Visual Function and Risk of Legal Blindness With Age in RPGR -Associated Retinal Degeneration: A
Bela J Parekh1,2, Joshua D Stein1,3, Rebhi Abuzaitoun1
1W.K. Kellogg Eye Center, Department of Ophthalmology and Visual Sciences, University of Michigan, Ann Arbor, Michigan, USA.
Clinical & Experimental Ophthalmology
|February 27, 2026
Summary
RPGR-associated retinal degeneration (RPGR-RD) causes progressive vision loss. Early intervention targeting the rod-cone phenotype before age 20 may be most effective for preserving visual field.
Area of Science:
- Ophthalmology
- Genetics
- Retinal Degeneration
Background:
- RPGR-associated retinal degeneration (RPGR-RD) is a genetic disorder affecting vision.
- Understanding the disease progression is crucial for developing effective treatments.
Purpose of the Study:
- To analyze the loss trajectory of best recorded visual acuity (BRVA) and Goldmann visual field (GVF) in patients with RPGR-RD.
- To identify potential windows for therapeutic intervention.
Main Methods:
- Retrospective analysis of longitudinal data from RPGR-RD patients.
- Classification of patients into rod-cone (RC), cone/cone-rod (CR), and female-carrier phenotypes.
- Scatterplots and local polynomial smoothers used to visualize BRVA and GVF decline over time.
Main Results:
- 281 patients (220 males, 61 female carriers) were included.
- The rod-cone (RC) phenotype showed a steep decline in GVF after age 20, with 53% legal blindness by GVF.
- The cone/cone-rod (CR) phenotype experienced legal blindness by BRVA at a median age of 60 years.
Conclusions:
- All RPGR-RD phenotypes exhibit progressive loss of BRVA and GVF.
- Interventions for the RC phenotype may be most effective before age 20, coinciding with rapid GVF decline.
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