Classification of Non-Infectious and/or Immune Mediated Choroiditis: A Brief Overview of the Essentials

Abstract

The choroid was poorly accessible to imaging investigation until the last decade of the last century. With the availability of more precise imaging methods such as indocyanine green angiography (ICGA) and, later, optical coherence tomography (OCT), enhanced depth OCT (EDI-OCT), and OCT angiography (OCTA), appraisal of choroidal inflammation has substantially gained in accuracy. This allowed to precisely determine which structures were touched in the different non-infectious choroiditis entities and made it possible to classify this group of diseases, ICGA signs, mainly hypofluorescent lesions, were identified and described. Previous publications have divided angiographic findings into two main sets of signs: (1) irregular "geographic" hypofluorescent areas corresponding to choriocapillaris non-perfusion and (2) round more regular, hypofluorescent dark dots more evenly distributed in the fundus corresponding to more deep choroidal stromal foci. These distinct findings allowed to subdivide and classify choroiditis into choriocapillaritis and stromal choroiditis. Additional signs were identified from EDI-OCT and OCTA examination supporting the classification of choroiditis into choriocapillaritis and stromal choroiditis. Results: Diseases involving principally the choriocapillaris included Multiple Evanescent White Dot Syndrome (MEWDS), Acute Posterior Multifocal Placoid Pigment Epitheliopathy (APMPPE), Idiopathic Multifocal Choroiditis (MFC), and Serpiginous Choroiditis (SC) as well as mixed forms. Diseases primarily involving the choroidal stroma included HLA-A29 Birdshot Retinochoroiditis (BRC), Vogt-Koyanagi-Harada disease (VKH), Sympathetic Ophthalmia (SO), and Sarcoidosis chorioretinitis (SARC). Thanks to new imaging investigations of the choroid, it is now possible to classify and understand the diverse clinicopathological mechanisms in the group of non-infectious choroiditis entities.