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

  • Ester Carreño*
  • , Panayiotis Maghsoudlou
  • , Alejandro Fonollosa
  • , Inês Leal
  • , Ariel Schlaen
  • , Alan Roy Abraham
  • , Andrew D Dick
  • , Aniruddha Agarwal
  • , Sapna Gangaputra
  • , Alessandro Invernizzi
  • , Amani Fawzi
  • , Elisabetta Miserocchi
  • , Rupesh Agrawal
  • , Douglas A Jabs
  • , David Sarraf
  • , Vishali Gupta*
  • ,
  • *Corresponding author for this work

Research output: Contribution to journalArticleAcademicpeer-review

Abstract

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.

Original languageEnglish
Pages (from-to)38-51
Number of pages14
JournalAmerican Journal of Ophthalmology
Volume278
DOIs
Publication statusPublished - Oct 2025

Keywords

  • Acute Disease
  • Coloring Agents/administration & dosage
  • Consensus
  • Fluorescein Angiography/methods
  • Humans
  • Indocyanine Green/administration & dosage
  • Multimodal Imaging/standards
  • Retinal Diseases/diagnosis
  • Retinal Pigment Epithelium/pathology
  • Tomography, Optical Coherence/methods
  • Uveitis/diagnosis

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