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Evidence and Consensus-Based Imaging Guidelines in Acute Posterior Multifocal Placoid Pigment Epitheliopathy (APMPPE)
Ester Carreño1, Panayiotis Maghsoudlou2, Alejandro Fonollosa3
1From the Hospital Universitario La Paz (E.C.), Madrid, Spain.
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.
Insights
Multimodal imaging (MUV) guidelines aid in diagnosing acute posterior multifocal placoid pigment epitheliopathy (APMPPE). These consensus-based recommendations improve disease activity assessment and clinical management.
Area of Science:
- Ophthalmology
- Medical Imaging
- Retinal Diseases
Background:
- Acute posterior multifocal placoid pigment epitheliopathy (APMPPE) is an inflammatory condition affecting the retina.
- Accurate diagnosis and monitoring of APMPPE are crucial for effective management.
- Multimodal imaging plays a key role in evaluating retinal disorders.
Purpose of the Study:
- To establish consensus-based guidelines for the use and interpretation of multimodal imaging in APMPPE.
- To standardize the diagnostic approach for APMPPE using various imaging modalities.
- To enhance the understanding of APMPPE activity and complications through imaging.
Main Methods:
- Literature review and consensus agreement using a nominal group technique (NGT).
- Expert committee reviewed representative APMPPE cases.
- Guidelines developed for diagnosis and follow-up using CFP, OCT, FFA, ICGA, FAF, and OCTA.
Main Results:
- Distinctive imaging findings characterize active APMPPE, including specific OCT and CFP features.
- Choriocapillaris non-perfusion on FFA, ICGA, or OCTA is critical for diagnosis.
- APMPPE activity can be classified into four stages: choroidal, chorioretinal, transitional, and resolved.
Conclusions:
- Multimodal imaging (MUV) criteria enhance the diagnosis of APMPPE, complementing existing classifications like SUN.
- These guidelines offer a framework for assessing disease activity and complications.
- Improved diagnostic accuracy and guided clinical management are expected outcomes.

