Monitoring Acute Posterior Multifocal Placoid Pigment Epitheliopathy Disease Progression Using Non-invasive Multimodal Imaging: A Case Series

TL;DR

This case series shows how non-invasive imaging can track the progression and treatment response of a rare eye disease called APMPPE.

Contribution

The study demonstrates the use of non-invasive multimodal imaging to monitor APMPPE without dye-based angiography.

Findings

Non-invasive imaging showed reduced hyper-reflectivity in retinal layers as the disease resolved.

Changes in autofluorescence and choriocapillaris flow voids correlated with clinical improvement.

Imaging biomarkers can detect disease activity and treatment effects in APMPPE.

Abstract

In this case series, we present two cases of acute posterior multifocal placoid pigment epitheliopathy (APMPPE): a patient presenting for the first time with headaches and prodromal symptomatology associated with mild photophobia who was later diagnosed with APMPPE, and a patient who presented with a relapse of the same disease entity who was recently started on mycophenolate mofetil in view of previous macular involvement. In both cases, disease progression and therapeutic effect were assessed using non-invasive imaging, including optical coherence tomography (OCT) and fundus photography. Imaging biomarkers in keeping with disease resolution included a reduction in hyper-reflectivity in the outer plexiform layer (OPL) and outer nuclear layer (ONL) overlying the active placoid lesions on OCT, a progression from heterogeneous autofluorescent foci to smaller hypo-autofluorescent ones demonstrating a reduction in the outer retinal infiltrate and emergent retinal pigment epithelium dysfunction as well as a reduction in choriocapillaris flow voids on OCT angiography as the clinical picture improved. This case series and accompanying literature review help demonstrate the use of non-invasive imaging modalities in monitoring disease progression and screening for new disease activity in APMPPE without requiring the use of frequent dye-based angiography studies.

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