Evidence and Consensus-Based Imaging Guidelines in Acute Posterior Multifocal Placoid Pigment Epitheliopathy (APMPPE) - Multimodal imaging in Uveitis (MUV) Taskforce Report 7.

PURPOSE

To develop consensus-based guidelines on use and interpretation of multimodal imaging in acute posterior multifocal placoid pigment epitheliopathy (APMPPE).

DESIGN

Consensus agreement led by literature, and an expert committee using a nominal group technique (NGT).

METHODS

The expert committee for APMPPE performed a thorough review of representative cases of APMPPE. The cases were used to develop guidelines for the diagnosis and follow-up of APMPPE using color fundus photography (CFP), optical coherence tomography (OCT), fundus fluorescein angiography (FFA), indocyanine green angiography (ICGA), fundus autofluorescence (FAF), and OCT angiography (OCTA). Structured NGT-based discussions were used to achieve consensus-based recommendations on imaging characteristics, disease activity, and complications, and subsequently were adopted by a vote of the entire taskforce.

RESULTS

Diagnosis of active APMPPE is characterized by distinctive imaging findings on CFP, and hyper-reflectivity of the ellipsoid zone (EZ), external limiting membrane (ELM), and outer nuclear layer (ONL) on OCT. Choriocapillaris non-perfusion, detectable via early-phase FFA, ICGA or OCTA, is crucial. In the early stages of APMPPE, OCT findings may be unremarkable, making FFA, ICGA, and/or OCTA relevant for the diagnosis. Based on the imaging findings, APMPPE can be classified into 4 stages of activity: choroidal, chorioretinal, transitional, and resolved. Following diagnosis, OCT and OCTA can be used to monitor lesion activity and identify potential complications.

CONCLUSIONS

MUV imaging criteria enable the identification of key diagnostic features for APMPPE, extending the Standardization of Uveitis Nomenclature (SUN) classification. These consensus-based guidelines provide a framework for evaluating disease activity and complications, enhancing diagnostic accuracy and guiding clinical management.