Evidence and Consensus-Based Imaging Guidelines in Acute Posterior Multifocal Placoid Pigment Epitheliopathy (APMPPE) - Multimodal imaging in Uveitis (MUV) Taskforce Report 7 - 13/09/25
Doi : 10.1016/j.ajo.2025.06.007
Ester Carreño 1, ⁎ 
, Panayiotis Maghsoudlou 2, Alejandro Fonollosa 3, 4, Inês Leal 5, 6, Ariel Schlaen 7, Alan Roy Abraham 8, Andrew D. Dick 8, 9, 10, 11, Aniruddha Agarwal 12, 13, Sapna Gangaputra 14, Alessandro Invernizzi 15, 16, Amani Fawzi 17, Elisabetta Miserocchi 18, Rupesh Agrawal 19, 20, 21, 22, Douglas A. Jabs 23, 24, David Sarraf 25, Vishali Gupta 26, ⁎
on behalf of the
, Panayiotis Maghsoudlou 2, Alejandro Fonollosa 3, 4, Inês Leal 5, 6, Ariel Schlaen 7, Alan Roy Abraham 8, Andrew D. Dick 8, 9, 10, 11, Aniruddha Agarwal 12, 13, Sapna Gangaputra 14, Alessandro Invernizzi 15, 16, Amani Fawzi 17, Elisabetta Miserocchi 18, Rupesh Agrawal 19, 20, 21, 22, Douglas A. Jabs 23, 24, David Sarraf 25, Vishali Gupta 26, ⁎ on behalf of the
Multimodal Imaging in Uveitis (MUV) Taskforce†
Alejandra de-la-Torre 27, Alejandro Fonollosa 28, Alessandro Invernizzi 29, 30, Amani Fawzi 31, Andrew Dick 32, 33, 34, 35, Aniruddha Agarwal 36, 37, Anita Agarwal 38, 39, Antoine Brezin 40, Ariel Schlaen 41, Bahram Bodaghi 42, Claudia Fabiani 43, David Sarraf 44, Debra A. Goldstein 45, Dilraj Grewal 46, Douglas A. Jabs 47, 48, Edmund Tsui 49, Elisabetta Miserocchi 50, 51, Ester Carreño 52, Francesco Pichi 36, Ilknur Tugal-Tutkun 53, Inês Leal 54, 55, Janet L. Davis 56, Jeannette Ossewaarde-van Norel 57, Jennifer E. Thorne 58, Jessica G. Shantha 59, Jose S. Pulido 60, Justine R. Smith 61, Jyotirmay Biswas 62, K. Bailey Freund 63, 64, Kathryn Pepple 65, Lee M. Jampol 45, Luca Cimino 66, 67, Marc De Smet 68, Maria Vittoria Cicinelli 69, Marion R. Munk 70, Massimo Accorinti 71, Maura Di Nicola 72, Meghan Berkenstock 58, Michael Altaweel 73, 74, Phoebe Lin 75, Quan Dong Nguyen 76, Richard Spaide 63, Rupesh Agrawal 77, 78, 79, Sapna S. Gangaputra 80, Soon Phaik Chee 81, Srinivas Sadda 82, Sumit Sharma 75, Timothy M. Janetos 45, Vishali Gupta 83, Zohar Habot-Wilner 84
27 Neuroscience Research Group (NEUROS), Neurovitae Center for Neuroscience, Institute of Translational Medicine (IMT), School of Medicine and Health Sciences, Universidad del Rosario, Bogotá, Colombia
28 Department of Ophthalmology, Biocruces Bizkaia Health Research Institute, Cruces University Hospital, University of the Basque Country, Barakaldo, Spain
29 Eye Clinic, Department of Biomedical and Clinical Science, Luigi Sacco Hospital, University of Milan, Milan, Italy
30 Department of Ophthalmology, Save Sight Institute, University of Sydney, Sydney, Australia
31 Northwestern University Feinberg School of Medicine, Chicago, USA
32 University of Bristol, UK
33 Bristol Eye Hospital, UK
34 Institute of Ophthalmology, University College London, London, UK
35 National Institute for Health Research Biomedical Research Centre, Moorfields Eye Hospital, London, UK
36 Eye Institute, Cleveland Clinic, Abu Dhabi, United Arab Emirates
37 Cleveland Clinic Lerner College of Medicine, Cleveland, Ohio, USA
38 California Pacific Medical Center (CPMC), San Francisco, CA, USA
39 West Coast Retina, San Francisco, CA, USA
40 Department of Ophthalmology, Ophtalmopôle, Hôpital Cochin, Assistance Publique-Hôpitaux de Paris, AP-HP, Université Paris Cité, 75014, Paris, France
41 Hospital Universitario Austral, Derqui-Pilar, Argentina
42 Department of Ophthalmology, IHU FOReSIGHT, Pitié-Salpêtrière Universtiy Hospital, Paris, France
43 Ophthalmology Unit, Department of Medicine, Surgery and Neurosciences, University of Siena and Azienda Ospedaliero-Universitaria Senese [European Reference Network (ERN) for Rare Immunodeficiency, Autoinflammatory, and Autoimmune Diseases (RITA) Center], Sienna, Italy
44 Stein Eye Institute, University of California, Los Angeles, California, USA
45 Department of Ophthalmology, Northwestern University Feinberg School of Medicine, Chicago, Illinois USA
46 Department of Ophthalmology, Duke University School of Medicine, Durham, NC, USA
47 Center for Clinical Trials and Evidence Synthesis, Department of Epidemiology, The Johns Hopkins Bloomberg School of Public Health, Baltimore, MD, USA
48 Wilmer Eye Institute, Department of Ophthalmology, The Johns Hopkins University School of Medicine, Baltimore, MD, USA
49 Stein Eye Institute, David Geffen School of Medicine at UCLA, Los Angeles, CA, USA
50 School of Medicine, Vita-Salute San Raffaele University, Milan, Italy
51 Department of Ophthalmology, IRCCS San Raffaele Scientific Institute, Milan, Italy
52 Hospital Universitario La Paz, Madrid, Spain
53 Department of Ophthalmology, Istanbul University, Istanbul, Turkey
54 Department of Ophthalmology, Hospital de Santa Maria, Unidade Local de Saúde (ULS) Santa Maria, Lisbon, PRT
55 Rheumatology Research Unit and Centro de Estudos Ciências da Visão, Faculdade de Medicina, Universidade de Lisboa, Lisbon, Portugal
56 Department of Ophthalmology, University of Miami Miller School of Medicine, Bascom Palmer Eye Institute, Miami, FL, USA
57 University Medical Center Utrecht, Utrecht, the Netherlands
58 Wilmer Eye Institute, The Johns Hopkins University School of Medicine, Baltimore, Maryland, USA
59 Francis I. Proctor Foundation, Department of Ophthalmology, University of California, San Francisco, USA
60 Wills Eye Hospital, Thomas Jefferson University Philadelphia, PA
61 College of Medicine and Public Health, Flinders University, Adelaide, South Australia, Australia
62 Uveitis & Ocular Pathology Department, Sankara Nethralaya, Chennai, India
63 Vitreous Retina Macula Consultants of New York, New York, USA
64 Department of Ophthalmology, NYU Grossman School of Medicine, New York, NY, USA
65 Department of Ophthalmology, University of Washington, Seattle, USA
66 Ocular Immunology Unit, Azienda USL-IRCCS di Reggio Emilia, Reggio Emilia, Italy
67 Department of Surgery, Medicine, Dentistry and Morphological Sciences, with Interest in Transplants, Oncology and Regenerative Medicine, University of Modena and Reggio Emilia, Modena, Italy
68 Micro Invasive Ocular Surgery Clinic, Lausanne, Switzerland
69 Department of Ophthalmology, IRCCS San Raffaele Scientific Institute, Milan, Italy
70 Augenarzt Praxisgemeinschaft Gutblick, Pfäffikon, Switzerland
71 IRCSS Fondazione Bietti, Rome, Italy
72 Bascom Palmer Eye Institute, University of Miami Miller School of Medicine, Miami, FL, USA
73 Department of Ophthalmology and Visual Sciences, University of Wisconsin, Madison, WI, USA
74 McPherson Eye Research Institute, University of Wisconsin, Madison, WI, USA
75 Cleveland Clinic Foundation, Cole Eye Institute, Cleveland, USA
76 Spencer Center for Vision Research, Byers Eye Institute, Stanford University, Palo Alto, CA
77 National Healthcare Group Eye institute, Tan Tock Seng Hospital, Singapore
78 LKC School of Medicine, Nanyang Technological University, Singapore
79 Duke NUS Medical School, Singapore
80 Vanderbilt Eye Institute, Vanderbilt University Medical Center, Nashville, TN, USA
81 Department of Ocular Inflammation and Immunology, Singapore National Eye Centre, Singapore
82 Doheny Eye Institute, University of California – Los Angeles, Los Angeles, California, USA
83 Advanced Eye Centre, Postgraduate Institute of Medical Education and Research, Chandigarh, India
84 Tel Aviv Medical Center, Tel Aviv University, Tel Aviv, Israel
1 From the Hospital Universitario La Paz (E.C.), Madrid, Spain
2 Department of Ophthalmology (P.M.), Cheltenham General Hospital NHS Foundation Trust, Cheltenham, UK
3 Department of Ophthalmology (A.F.), Biocruces Bizkaia Health Research Institute, Cruces University Hospital, University of the Basque Country, Barakaldo, Spain
4 Department of Retina (A.F.), Instituto Oftalmológico Bilbao, Bilbao, Spain
5 Department of Ophthalmology (I.L.), Hospital de Santa Maria, Unidade Local de Saúde (ULS) Santa Maria, Lisbon, Portugal
6 Rheumatology Research Unit and Centro de Estudos Ciências da Visão (I.L.), Faculdade de Medicina, Universidade de Lisboa, Lisbon, Portugal
7 Department of Ophthalmology (A.S.), Hospital Universitario Austral, Derqui-Pilar, Argentina
8 University of Bristol (A.R.A., A.D.D.), Bristol Eye Hospital, UK
9 Academic Unit of Ophthalmology (A.D.D.), Bristol Medical School, University of Bristol, Bristol, UK
10 Institute of Ophthalmology (A.D.D.), University College London, London, UK
11 National Institute for Health Research Biomedical Research Centre (A.D.D.), Moorfields Eye Hospital, London, UK
12 The Eye Institute (A.A.), Cleveland Clinic Abu Dhabi, Abu Dhabi, United Arab Emirates
13 Cleveland Clinic Lerner College of Medicine (A.A.), Cleveland, Ohio, USA
14 Vanderbilt Eye Institute (S.G.), Vanderbilt University Medical Center, Nashville, Tennessee, USA
15 Eye Clinic (A.I.), Department of Biomedical and Clinical Science, Luigi Sacco Hospital, University of Milan, Milan, Italy
16 Department of Ophthalmology (A.I.), Save Sight Institute, University of Sydney, Sydney, Australia
17 Department of Ophthalmology (A.F.), Northwestern University Feinberg School of Medicine, Chicago, Illinois, USA
18 Division of Head and Neck (E.M.), Ophthalmology Unit, San Raffaele Scientific Institute, School of Medicine, Vita-Salute San Raffaele University, Milan, Italy
19 National Healthcare Group Eye institute (R.A.), Tan Tock Seng Hospital, Singapore
20 LKC School of Medicine (R.A.), Nanyang Technological University, Singapore
21 Duke NUS Medical School (R.A.), Singapore
22 Ocular Infections and Anti-Microbials (R.A.), Singapore Eye Research Institute, Singapore
23 Center for Clinical Trials and Evidence Synthesis (D.A.G.), Department of Epidemiology, The Johns Hopkins Bloomberg School of Public Health, Baltimore, Maryland, USA
24 Wilmer Eye Institute (D.A.G.), Department of Ophthalmology, The Johns Hopkins University School of Medicine, Baltimore, Maryland, USA
25 Stein Eye Institute (D.S.), University of California, Los Angeles, California, USA
26 Advanced Eye Centre (V.G.), Postgraduate Institute of Medical Education and Research, Chandigarh, Punjab, India
⁎Inquiries to Vishali Gupta, MD, Advanced Eye Center, Post Graduate Institute of Medical Education and Research (PGIMER), Chandigarh, PB, India.Advanced Eye CenterPost Graduate Institute of Medical Education and Research (PGIMER)ChandigarhPBIndia⁎⁎Inquiries to Ester Carreño, MD, Hospital Universitario La Paz, Fuencarral-El Pardo, Madrid, Spain.Hospital Universitario La PazFuencarral-El PardoMadridSpain
Résumé |
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.
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| Supplemental Material available at AJO.com. |
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| This invited article was submitted as part of the Virtual Special Issue on Multimodal Imaging of Non-Infectious Posterior Uveitis by the The International Uveitis Study Group MUV Working Group. |
© 2025
The Authors. Publié par Elsevier Masson SAS. Tous droits réservés.
Vol 278
P. 38-51 - octobre 2025 Retour au numéro
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