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Diagnostics
Special Issues
Non-infectious and/or Immune Mediated Choroiditis, Clinical Entities, Diagnosis and Management
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Non-infectious and/or Immune Mediated Choroiditis, Clinical Entities, Diagnosis and Management

A special issue of Diagnostics (ISSN 2075-4418). This special issue belongs to the section "Optical Diagnostics".

Deadline for manuscript submissions: closed (1 December 2021) | Viewed by 15894

Special Issue Editors

Dr. Carl P. Herbort Jr

E-Mail Website
Guest Editor
Retinal and Inflammatory Eye Diseases, Centre for Ophthalmic Specialized Care (COS), Lausanne, Switzerland
Interests: non-infectious choroiditis; choroidal inflammation; choriocapillaritis; stromal choroiditis; MEWDS; APMPPE; idiopathic multifocal choroiditis; serpiginous choroiditis; HLA-A29 birdshot retinochoroiditis; Vogt–Koyanagi–Harada disease; sympathetic ophthalmia; ocular sarcoidosis; choroidal lesions in systemic vasculitis; indocyanine green angiography; optical coherence tomography; enhanced depth imaging OCT; corticosteroids; immunosuppressive therapy
Special Issues, Collections and Topics in MDPI journals
Prof. Ilknur Tugal-Tutkun

E-Mail Website
Guest Editor
Department of Ophthalmology, University of Istanbul, Istanbul, Turkey
Interests: non-infectious choroiditis; choroidal inflammation; choriocapillaritis; stromal choroiditis; MEWDS; APMPPE; idiopathic multifocal choroiditis; serpiginous choroiditis; HLA-A29 birdshot retinochoroiditis; Vogt–Koyanagi–Harada disease; sympathetic ophthalmia; ocular sarcoidosis; choroidal lesions in systemic vasculitis; indocyanine green angiography; optical coherence tomography; enhanced depth imaging OCT; corticosteroids; immunosuppressive therapy

Special Issue Information

Dear Colleagues,

Non-infectious choroiditis is a group of uveitis entities that have been poorly understood until recently, because precise exploration of choroidal inflammation was not possible. This changed when indocyanine green angiography (ICGA) came into use in the late 1990s, and/or enhanced depth imaging optical coherence tomography (EDI-OCT) in 2008 became available. Thanks to these investigational imaging methods, it became possible to classify and understand the diverse pathological mechanisms of non-infectious choroiditis entities. These novel investigative methodologies, as well as others, allowed subdividing non-infectious choroiditis into diseases principally affecting the choriocapillaris, i.e., choriocapillaritis diseases, and those principally affecting the choroidal stroma, i.e., stromal choroiditis entities. This opened a way toward precise appraisal and follow-up of choroidal inflammatory diseases.

The aim of this project is to give a global and comprehensive approach to this relatively recently described group of diseases. We expect to receive excellent reviews and original articles on the anatomy and the immunology of the choroid, novel investigative imaging modalities, choriocapillaris entities, and stromal diseases.

Dr. Carl P. Herbort Jr
Prof. Ilknur Tugal-Tutkun
Guest Editors

Manuscript Submission Information

Manuscripts should be submitted online at www.mdpi.com by registering and logging in to this website. Once you are registered, click here to go to the submission form. Manuscripts can be submitted until the deadline. All submissions that pass pre-check are peer-reviewed. Accepted papers will be published continuously in the journal (as soon as accepted) and will be listed together on the special issue website. Research articles, review articles as well as short communications are invited. For planned papers, a title and short abstract (about 100 words) can be sent to the Editorial Office for announcement on this website.

Submitted manuscripts should not have been published previously, nor be under consideration for publication elsewhere (except conference proceedings papers). All manuscripts are thoroughly refereed through a single-blind peer-review process. A guide for authors and other relevant information for submission of manuscripts is available on the Instructions for Authors page. Diagnostics is an international peer-reviewed open access semimonthly journal published by MDPI.

Please visit the Instructions for Authors page before submitting a manuscript. The Article Processing Charge (APC) for publication in this open access journal is 2600 CHF (Swiss Francs). Submitted papers should be well formatted and use good English. Authors may use MDPI's English editing service prior to publication or during author revisions.

Keywords

  • Anatomy and immunology of the choroid
  • Classification of non-infectious choroiditis
  • Imaging investigation of choroiditis
  • New treatment paradigms of non-infectious choroiditis

Published Papers (3 papers)

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Review

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50 pages, 26798 KiB  
Review
Classification of Non-Infectious and/or Immune Mediated Choroiditis: A Brief Overview of the Essentials
by Carl P. Herbort , Jr., Alessandro Mantovani, Ilknur Tugal-Tutkun and Ioannis Papasavvas
Diagnostics 2021, 11(6), 939; https://doi.org/10.3390/diagnostics11060939 - 24 May 2021
Cited by 18 | Viewed by 5038
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 [...] Read more.
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. Full article
(This article belongs to the Special Issue Non-infectious and/or Immune Mediated Choroiditis, Clinical Entities, Diagnosis and Management)
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15 pages, 4965 KiB  
Perspective
HLA-A29 Birdshot Retinochoroiditis in Its 5th Decade: Selected Glimpses into the Intellectual Meanderings and Progresses in the Knowledge of a Long-Time Misunderstood Disease
by Marina Papadia, Carlos Pavésio, Christine Fardeau, Piergiorgio Neri, Philippe G. Kestelyn, Ioannis Papasavvas and Carl P. Herbort
Diagnostics 2021, 11(7), 1291; https://doi.org/10.3390/diagnostics11071291 - 19 Jul 2021
Cited by 10 | Viewed by 3827
Abstract
The appraisal of HLA-A29 birdshot retinochoroiditis (BRC) was fraught with pitfalls and misunderstandings. Progress in investigational methods has led to better knowledge and management of the disease. Our aim was to assess some of the steps that have led to better characterisation of [...] Read more.
The appraisal of HLA-A29 birdshot retinochoroiditis (BRC) was fraught with pitfalls and misunderstandings. Progress in investigational methods has led to better knowledge and management of the disease. Our aim was to assess some of the steps that have led to better characterisation of the clinical entity of BRC. We performed a literature search analysing the relevant progress in disease origin, investigational and imaging methods, clinicopathology and classification, diagnostic criteria and management. Following developments were judged essential in the better appraisal and understanding of the disease: (1) new immunopathological hypotheses regarding the role of endoplasmic reticulum peptidases, (2) the essential importance of HLA testing, (3) relevant imaging modalities among which indocyanine green angiography is crucial, (4) diagnostic criteria that allow early diagnosis and (5) need of an early prolonged, as well as aggressive treatment combining more than one immunosuppressive agent. Based on these findings it is now possible to better define BRC, an indolent however severe disease, unlike thought before, involving the choroidal stroma and the retina independently and concomitantly that can be diagnosed early thanks to indocyanine green angiography and should be treated early and relentlessly. Full article
(This article belongs to the Special Issue Non-infectious and/or Immune Mediated Choroiditis, Clinical Entities, Diagnosis and Management)
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16 pages, 11134 KiB  
Case Report
Acute Zonal Occult Outer Retinopathy (AZOOR) Results from a Clinicopathological Mechanism Different from Choriocapillaritis Diseases: A Multimodal Imaging Analysis
by Carl P. Herbort, Jr., Ilir Arapi, Ioannis Papasavvas, Alessandro Mantovani and Bruno Jeannin
Diagnostics 2021, 11(7), 1184; https://doi.org/10.3390/diagnostics11071184 - 29 Jun 2021
Cited by 10 | Viewed by 6180
Abstract
Background and aim: AZOOR is a rare disease characterized by loss of zones of outer retinal function, first described by J Donald Gass in 1993. Symptoms include acute onset photopsias and subjective visual field losses. The syndrome is characterized by a normal fundus [...] Read more.
Background and aim: AZOOR is a rare disease characterized by loss of zones of outer retinal function, first described by J Donald Gass in 1993. Symptoms include acute onset photopsias and subjective visual field losses. The syndrome is characterized by a normal fundus appearance, scotomas and electroretinographic changes pointing towards outer retinal dysfunction. Evolution, response to immunosuppressive treatment and outcome are difficult to predict. The aim of this small case series was to identify the morphological changes and sequence of events in AZOOR thanks to multimodal imaging. Methods: Charts of AZOOR patients seen in the Centre for Ophthalmic Specialized care (COS, Lausanne, Switzerland) were analyzed by multimodal imaging including fundus photography, fluorescein angiography (FA), indocyanine green angiography (ICGA), blue light fundus autofluorescence (BL-FAF) and spectral domain optical coherence tomography (SD-OCT) in addition to a complete ophthalmological examination including visual field testing and microperimetry, as well as OCT angiography (OCT-A) and ganglion-cell complex analysis when available. Cases and Results: Three AZOOR patients with a mean follow-up of 47 ± 25.5 months were included following the clinical definitions laid down by J Donald Gass. The primary damage was identified at the level of the photoreceptor outer segments with an intact choriocapillaris and retinal pigment epithelium (RPE) layer, these structures being only secondarily involved with progression of the disease. Conclusion: Although AZOOR has often been included within white dot syndromes, some of which are now known to be choriocapillaris diseases (choriocapillaritis entities), our findings clearly commend to differentiate AZOOR from entities such as MEWDS (Multiple evanescent white dot syndrome), APMPPE (Acute Posterior Multifocal Placoid Pigment Epitheliopathy), MFC (Multifocal Choroiditis) and others, as the damage to photoreceptors is primary in AZOOR (a retinopathy) and secondary in choriocapillaritis (a choriocapillaropathy). Full article
(This article belongs to the Special Issue Non-infectious and/or Immune Mediated Choroiditis, Clinical Entities, Diagnosis and Management)
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