Non-Infectious Choroiditis Subdivided into Its Diverse Pathophysiological Sub-Groups Is the Best-Known and Most Appropriate Nomenclature to Date for the Reclassification and Diagnosis of Former White Dot Entities

Abstract

White dot syndromes (WDS), a purely descriptive term formulated in 1995, grouped inflammatory conditions primarily affecting the choroid that shared the clinical appearance of white dots on fundus examination, thus presenting similar white dots on fundus examination, which, however, had diverse pathophysiologies and different primary localizations of inflammation that did not warrant their association. Some of them, including birdshot retinochoroiditis (BRC) and sympathetic ophthalmia, produced predominant inflammation in the choroidal stroma, while others, including multiple evanescent white dot syndrome (MEWDS) and acute posterior multifocal placoid pigment epitheliopathy (APMPPE), caused inflammatory non-perfusion of the choriocapillaris. The aim of this review is to build upon a pathophysiology-based classification framework that groups these entities under the broader concept of non-infectious choroiditis and subdivides them according to their predominant inflammatory mechanisms and anatomical localization. This framework distinguishes diseases characterized by inflammatory occlusion of the choriocapillaris with consequent perfusion dysfunction, including MEWDS, APMPPE, multifocal choroiditis (MFC), and serpiginous choroiditis, from conditions primarily involving inflammatory infiltration of the choroidal stroma, such as birdshot retinochoroiditis (BRC), Vogt-Koyanagi-Harada disease (VKH), and sympathetic ophthalmia. Moreover, the review raises the question of how such an inadequate term, based on unscientific assumptions, could gain such quick access to ophthalmic practice and literature, be immediately adopted in major textbooks, and persist for so long being unrecognized as a misnomer. The slow and progressive reevaluation of this nomenclature over more than two decades is related and contextualized.