Pubblicazioni

Autori:

Antropoli, Alessio; Bianco, Lorenzo; Romano, Francesco; Trinco, Andrea; Arrigo, Alessandro; Benadji, Amine; Atia, Raphaël; Palacci, Oana; Dagostinoz, Dorothée; Devisme, Céline; Condroyer, Christel; Antonio, Aline; Bosello, Francesca; Casati, Stefano; Salvetti, Anna Paola; Zaffalon, Chiara; Gaudric, Alain; Sahel, José-Alain; Staurenghi, Giovanni; Bandello, Francesco; Sennlaub, Florian; Zeitz, Christina; Meunier, Isabelle; Battaglia Parodi, Maurizio; Audo, Isabelle

Titolo:

Extensive Macular Atrophy with Pseudodrusen-like appearance (EMAP) Clinical characteristics, diagnostic criteria, and insights from allied Inherited Retinal Diseases and Age-related Macular Degeneration

Anno:

2025

Tipologia prodotto:

Articolo in Rivista

Tipologia ANVUR:

Articolo su rivista

Lingua:

Inglese

Formato:

Elettronico

Referee:

Sì

Nome rivista:

PROGRESS IN RETINAL AND EYE RESEARCH

ISSN Rivista:

1350-9462

N° Volume:

104

Numero o Fascicolo:

101320

Intervallo pagine:

1-43

Parole chiave:

Age-related macular degeneration; Diffuse-Trickling Geographic atrophy; Extensive macular atrophy with pseudodrusen-like appearance; Inherited retinal diseases; Multimodal imaging; Retinal electrophysiology

Breve descrizione dei contenuti:

Extensive macular atrophy with pseudodrusen-like appearance (EMAP) was first described in France in 2009 as a symmetric and rapidly progressive form of macular atrophy primarily affecting middle-aged individuals. Despite the recent identification of a significant number of cases in Italy and worldwide, EMAP remains an underrecognized condition. The clinical triad typical of EMAP consists of vertically oriented macular atrophy with multilobular borders, pseudodrusen-like deposits across the posterior pole and mid-periphery, and peripheral pavingstone degeneration. Nonetheless, recent research has portrayed EMAP as a highly stage-dependent condition, allowing the identification of novel disease hallmarks, including a diffuse separation between the Bruch's membrane and the retinal pigment epithelium, along with consistent sparing of a region temporal to the macula. Additionally, retinal electrophysiology is particularly useful in distinguishing EMAP from age-related macular degeneration (AMD). Supported by unpublished data from the largest EMAP cohorts worldwide, this review aims to provide a comprehensive and updated description of EMAP, now recognized as a severely blinding disease characterized by diffuse chorioretinal atrophy and photoreceptor dysfunction. Furthermore, we propose a set of diagnostic criteria that incorporate clinical, imaging, and functional tests, to facilitate the recognition of this clinical entity. Lastly, we aim to shed light on its pathogenesis by comparing it with AMD and monogenic retinal disorders exhibiting similar phenotypes.

Pagina Web:

https://doi.org/10.1016/j.preteyeres.2024.101320

Id prodotto:

143618

Handle IRIS:

11562/1148751

ultima modifica:

30 gennaio 2025

Citazione bibliografica:

Antropoli, Alessio; Bianco, Lorenzo; Romano, Francesco; Trinco, Andrea; Arrigo, Alessandro; Benadji, Amine; Atia, Raphaël; Palacci, Oana; Dagostinoz, Dorothée; Devisme, Céline; Condroyer, Christel; Antonio, Aline; Bosello, Francesca; Casati, Stefano; Salvetti, Anna Paola; Zaffalon, Chiara; Gaudric, Alain; Sahel, José-Alain; Staurenghi, Giovanni; Bandello, Francesco; Sennlaub, Florian; Zeitz, Christina; Meunier, Isabelle; Battaglia Parodi, Maurizio; Audo, Isabelle, Extensive Macular Atrophy with Pseudodrusen-like appearance (EMAP) Clinical characteristics, diagnostic criteria, and insights from allied Inherited Retinal Diseases and Age-related Macular Degeneration «PROGRESS IN RETINAL AND EYE RESEARCH» , vol. 104 , n. 101320 , 2025 , pp. 1-43

Consulta la scheda completa presente nel repository istituzionale della Ricerca di Ateneo