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.