Progressive Expansion of Chorioretinal Atrophy in Placoid-Spectrum Disease
Timothy M Boyce1, Elliott H Sohn1, Jonathan F Russell1
1Institute for Vision Research, Department of Ophthalmology and Visual Sciences, Carver College of Medicine, University of Iowa, Iowa, Iowa, USA.
Ocular Immunology and Inflammation
|May 1, 2026
Summary
Chorioretinal atrophy, common in placoid disorders like APMPPE and RPC/SC, can expand over time even when the disease is inactive. This expansion was observed in patients treated with immunomodulatory therapy.
Area of Science:
- Ophthalmology
- Retinal Diseases
- Immunosuppression Therapy
Background:
- Chorioretinal atrophy is a key feature of placoid-spectrum disorders, including APMPPE, RPC, and SC.
- The progression of atrophy and its behavior during disease quiescence are not well understood.
Purpose of the Study:
- To investigate the rate and evolution of chorioretinal atrophy in placoid-spectrum disorders.
- To determine if atrophy expands even when the disease is clinically inactive.
Main Methods:
- Retrospective chart review of 17 patients with placoid-spectrum disease.
- Multimodal imaging analysis to assess chorioretinal atrophy (RPE/outer retinal atrophy).
- Qualitative and quantitative analysis of atrophy progression during disease quiescence.
Main Results:
- Chorioretinal atrophy was present in 83.3% of RPC/SC patients and 20% of APMPPE patients.
- Atrophy expanded over time at a mean rate of 0.09 mm/year, irrespective of disease activity.
- Expansion of atrophy was noted even in patients achieving clinical quiescence on therapy.
Conclusions:
- Chorioretinal atrophy is more prevalent in RPC/SC compared to APMPPE.
- Atrophic lesions in placoid disorders can enlarge progressively, even when the underlying condition is quiescent under treatment.
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