Acute Posterior Multifocal Placoid Pigment Epitheliopathy Spectrum on Multimodal Imaging: A Severity-Driven Ischemic...

Area of Science:

• Ophthalmology
• Medical Imaging
• Retinal Diseases

Background:

• Acute Posterior Multifocal Placoid Pigment Epitheliopathy (APMPPE) is a rare inflammatory condition affecting the retina.
• Understanding the spectrum and imaging characteristics of APMPPE is crucial for diagnosis and management.
• Previous studies have described classic APMPPE and ampiginous choroiditis (AC), but the relationship between these entities remains debated.

Purpose of the Study:

• To define multimodal imaging patterns across the APMPPE spectrum.
• To correlate imaging features with the underlying pathogenesis of APMPPE and related conditions.
• To investigate the potential for a continuum of disease presentation.

Main Methods:

• Retrospective, observational, single-center study of patients with APMPPE-spectrum disease.
• Categorization into three patterns: classic APMPPE, peripheral APMPPE, and ampiginous choroiditis (AC).
• Multimodal imaging included color fundus photography (CFP), fundus autofluorescence (FAF), and indocyanine green angiography (ICGA).

Main Results:

• 19 eyes from 10 patients were analyzed, categorized into classic APMPPE (3 eyes), peripheral APMPPE (8 eyes), and AC (8 eyes).
• A hyperfluorescent peripheral vessel abutting plaques on late ICGA was observed in 9/19 eyes.
• Far-peripheral FAF hypoautofluorescence was present in all AC eyes, absent in classic APMPPE and peripheral APMPPE, indicating distinct patterns.

Conclusions:

• A distinct phenotypic pattern, peripheral APMPPE (PAPMPPE), was identified using multimodal imaging.
• PAPMPPE exhibits an intermediate phenotype between classic APMPPE and AC.
• These findings support the concept of a severity-graded continuum within the APMPPE spectrum.