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

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.