Mechanisms, Pathophysiology and Current Immunomodulatory/Immunosuppressive Therapy of Non-Infectious and/or Immune-Mediated Choroiditis

Abstract

Background: Non-infectious choroiditis comprises immune-mediated diseases resulting from diverse pathophysiological mechanisms. These conditions are sub-divided into two main groups, (1) diseases of the choriocapillaris and (2) diseases of the choroidal stroma. The purpose of this study is to expose the pathophysiology of the most common diseases of both these groups and recommend the optimal immunomodulatory/immunosuppressive therapy of each analyzed condition based on literature data and data from our own centers. Material and Methods: Narrative review. In the group of choriocapillaritis entities or primary inflammatory choriocapillaropathies (PICCPs) including multiple evanescent white dot syndrome (MEWDS), acute posterior multifocal placoid pigment epitheliopathy (APMPPE), idiopathic multifocal choroiditis (MFC) and serpiginous choroiditis (SC), as well as secondary choriocapillaritides including acute syphilitic posterior multifocal placoid chorioretinitis (ASPMPC) and tuberculosis-related SC (TB-SC), were analyzed. In the group of stromal choroidites, HLA-A29 birdshot retinochoroiditis (BRC) and Vogt-Koyanagi-Harada (VKH) disease were included. For each entity a literature search, in the PubMed database, on treatment was performed and analyzed and the therapeutic attitudes of our own centers were presented. Conclusion: Management of immune-mediated choroiditis implies vigorous immunosuppressive therapy given in a prompt and prolonged fashion in most of these entities.

Correlation Between Inflammatory Foci Reactivation and Atrophy Growth in Eyes With Idiopathic Multifocal Choroiditis

“…Because MFC affects primarily young women of childbearing age, 3,5,26,29 the findings of this study should be taken into account when evaluating the need for a treatment that could have serious side effects, especially during pregnancy. 31,32 Limitations of this study are mainly related to its retrospective design. It is possible that reactivations of MFC foci might have appeared and resolved in the interval between visits and therefore might not have been correctly identified, leading to underrepresentation of inflammatory relapses.…”

“…Because MFC affects primarily young women of childbearing age, 3,5,26,29 the findings of this study should be taken into account when evaluating the need for a treatment that could have serious side effects, especially during pregnancy. 31,32…”

A retrospective study describing the effective exchange of total blood plasma for disease control in the exacerbation of serpiginous choroiditis

“…Pathologically, there is loss of choroidal capillaries, and atrophy or loss and degeneration of the overlying retinal pigment epithelium. Two mechanisms have been hypothesized in the non-infectious setting: choriocapillaris nonperfusion due to choroidal capillaritis, and interstitial choroiditis due to an autoimmune reaction to interstitial antigens [5]. Anti-double-stranded DNA antibody (IU/mL) 1.9 <10…”

Multimodal Imaging of White Dot Syndromes

“…However, the clinical finding of white spots itself is variably present, the pathophysiology, as supported by multimodal imaging, may differ between them and the clinical course is different. For this reason, the term "noninfectious choroiditis" has been suggested to be more appropriate [1][2][3][4].…”