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This article provides an overview of Vogt-Koyanagi-Harada (VKH) disease, an autoimmune disorder that affects multiple systems in the body. It discusses the four clinical phases of the disease and emphasizes the importance of early diagnosis and treatment. The article also explores recent studies on diagnostic criteria, imaging features, and treatment options for VKH disease. It includes brief reports on atypical cases of the disease and discusses the use of OCT angiography and adalimumab therapy in monitoring and treating VKH disease. The article highlights the need for prompt diagnosis and early intervention to prevent complications and visual impairment. It also mentions a rare case of a 2-year-old girl with VKH disease and autoimmune polyendocrinopathy candidiasis ectodermal dystrophy syndrome, a rare genetic disease. The authors encourage further research on VKH disease. [Extracted from the article]

We present a 20-year-old woman who was diagnosed with subacute sclerosing panencephalitis (SSPE) 20 months after presenting with unilateral retinitis. At presentation, the patient had two inferotemporal macular lesions in her left eye. Corresponding to these areas, optical coherence tomography (OCT) showed hyporeflective spaces with loss of nearly all of the retinal layers. OCT-angiography (OCTA) demonstrated some flow deficit areas with a reduction in the vessel density. Her serum measles antibody titre was high (IgG >5000.0 mIU/ml). Twenty months later the macular lesions had diminished in size, and there was some focal retinal thinning with interruption of the ellipsoid zone. OCTA showed that the flow deficit areas were diminished in size together with the relatively improved perfusion density. Neurological examination disclosed myoclonic jerks. Neuropsychological assessment demonstrated impaired executive function, attention, and narrowed lexical fluency. Measles IgG antibody was high in the cerebrospinal fluid (>230.0 U/ml). Brain magnetic resonance imaging demonstrated bilateral, non-specific, small foci of T2 hyperintensity in the frontoparietal subcortical white matter and centrum semiovale. The present case is the first where OCTA findings of SSPE-related retinal lesions have been described. [ABSTRACT FROM AUTHOR]

To investigate the correlation between widefield fundus fluorescein angiography (WF-FA) and anterior chamber laser flare photometry (LFP-flare) in Behçet uveitis (BU). Patients with BU who underwent Heidelberg WF-FA between March 2016 and March 2018 were included. Demographics, ocular findings, LFP-flare, central macular thickness were retrospectively analyzed. Eighty-six patients (162 eyes) were included. There was a high correlation between WF-FA leakage score and LFP-flare (rho: 0.691, p <.001). For a flare value of 5.75 ph/ms or higher, the sensitivity for predicting angiographic leakage was 70% and the specificity was 94% (AUC: 0.897, p <.001). Flare values that were equal or above 7.6 ph/ms were found to be 100% specific for leakage in the WF-FA. A strong correlation was found between WF-FA leakage and LFP-flare in patients with BU. High LFP-flare levels indicate subclinical posterior segment inflammation in clinically inactive BU. [ABSTRACT FROM AUTHOR]

To report demographic and clinical profiles of children with uveitis in theTurkishpopulation. The data of the pediatric uveitis cases in the nation wide uveitis database were analyzed. The study included 697 eyes of 442 patients with a meanage of 10.8 ± 3.8 years. There were 333 patients (75.3%) with non-infectious uveitis and 69 patients (15.6%) with infectious uveitis. Pars planitis (20.1%) was leading clinical form followed by idiopathic cases with uveitis other than pars planitis (18.8%), juvenile idiopathic arthritis(JIA) related uveitis (12.4%), Behçet uveitis (9.3%) and toxoplasma retinochoroiditis (7.9%). Ocular involvement was unilateral in 187 patients (42.3%) and bilateral in 255 patients (57.7%). The most common anatomiclocation of uveitis was anterior uveitis (39.1%), followed by intermediate uveitis (29.4%), panuveitis (16.1%) and posterior uveitis (15.4%). The most common systemic association was JIA in the younger children and Behçet disease in the older children. [ABSTRACT FROM AUTHOR]

The editors of Ocular Immunology and Inflammation have published a special edition of their journal on pediatric uveitis, which they have named the "Disease of the Year for 2023." This edition includes contributions from various parts of the world, providing an international perspective on the epidemiology, patterns of disease, and treatment options for pediatric uveitis. The articles cover topics such as the demographic and clinical profiles of children with uveitis in different countries, the long-term outcomes and complications of non-infectious uveitis, and the use of different medications to treat the condition. The edition also includes case reports and case series highlighting interesting and unique cases related to pediatric uveitis. The editors express their gratitude to everyone involved in bringing this special edition to fruition and look forward to further advancements in understanding this complex disease in the coming year. [Extracted from the article]

To report the presenting features and outcomes in patients who developed bilateral acute iris transillumination (BAIT) or bilateral acute depigmentation of iris (BADI) following acute COVID-19 infection. Thirty two eyes of 16 patients were reviewed retrospectively. The severity of COVID-19 infection, use of antibiotics, time of onset of ocular symptoms; ocular signs, the course and surgical procedures were recorded. 24 eyes of 12 BAIT and eight eyes of four consecutive BADI patients were included. The mean time between infection and onset of ocular symptoms was 2.5±1.1 weeks. Nine patients were treated with oral moxifloxacin for COVID-19 prior to presentation. Trabeculectomy was performed in 7 eyes (21.8%) of 5 BAIT patients; in the postoperative follow-up, IOP was controlled without medication in 6 eyes, with medication in 1 eye. BADI and BAIT can also develop after COVID-19 infection. A significant proportion of BAIT patients may require glaucoma surgery. [ABSTRACT FROM AUTHOR]

This article discusses the impact of cytomegalovirus (CMV) on the eye, specifically CMV retinitis (CMVR) in immunocompromised patients. While CMVR is commonly associated with acquired immunodeficiency syndrome (AIDS), the proportion of HIV-negative patients with CMVR has been increasing due to factors such as immunosuppressive therapies, old age, diabetes, autoimmune conditions, and hematological abnormalities. The article highlights various reports on CMV and the eye, including cases of panretinal occlusive vasculitis, retinal neovascularization after resolution of CMVR, predictors for CMVR recurrence, risk factors for delayed diagnosis, and the impact of CMVR on quality of life. The authors also discuss the potential use of 2% ganciclovir drops in preventing relapses of Posner-Schlossman syndrome. Overall, the article aims to provide valuable insights for healthcare professionals in their daily practice. [Extracted from the article]

An effective therapy to reduce the number and severity of HLA-B27-related acute anterior uveitis (AAU) recurrences represents a clinical need. Curcumin is a promising therapeutic option in various inflammatory eye diseases. To enhance its absorption and eye tissue selectivity, a phospholipidic-curcumin complex (PHBC) has been formulated (Iphytoone®, Eye Pharma S.p.A.). This study investigates if PHBC is effective and safe to decrease the number and intensity of HLA-B27-related AAU relapses. HLA-B27-related AAU patients were randomly divided to receive PHBC or placebo for 12 months (NCT03584724). Compared with the previous year, the number of relapses decreased in both groups. The proportion of responders was significantly higher in the PBHC group. The severity of attacks was comparable. The study drug was well tolerated. A beneficial effect of PHBC treatment is suggested because the proportion of responders was significantly higher in this group of patients. [ABSTRACT FROM AUTHOR]

Background: The global and precise follow-up of uveitis has become possible with the availability of dual fluorescein (FA) and indocyanine green angiography (ICGA) since the mid-1990s. Progressively, additional non-invasive imaging methods have emerged, bringing value-added precision to the imaging appraisal of uveitis, including, among others, optical coherence tomography (OCT), enhanced-depth imaging OCT (EDI-OCT) and blue light fundus autofluorescence (BAF). More recently, another complementary imaging method, OCT-angiography (OCT-A), further allowed retinal and choroidal circulation to be imaged without the need for dye injection. Purpose: The purpose of this review was aimed at examining the evidence in published reports indicating whether OCT-A could possibly replace dye angiographic methods, as well as the real practical impact of OCT-A. Methods: A literature search in the PubMed database was performed using the terms OCT-angiography and uveitis, OCTA and uveitis and OCT-A and uveitis. Case reports were excluded. Articles were classified into technical reports, research reports and reviews. Articles in the two latter categories were analyzed in a more detailed, individual fashion. Special attention was paid to whether there were arguments in favor of an exclusive rather than complementary use of OCT-A. Furthermore, a synthesis of the main practical applications of OCT-A in the management of uveitis was attempted. Results: Between 2016 (the year of the first articles) and 2022, 144 articles containing the search terms were identified. After excluding case report articles, 114 articles were retained: 4 in 2016, 17 in 2017, 14 in 2018, 21 in 2019, 14 in 2020, 18 in 2021 and 26 in 2022. Seven articles contained technical information or consensus-based terminology. Ninety-two articles could be considered as clinical research articles. Of those, only two hinted in their conclusions that OCT-A could hypothetically replace dye methods. The terms mostly used to qualify the contribution of the articles in this group were "complementary to dye methods", "adjunct", "supplementing" and other similar terms. Fifteen articles were reviews, none of which hinted that OCT-A could replace dye methods. The situations where OCT-A represented a significant practical contribution to the practical appraisal of uveitis were identified. Conclusion: To date, no evidence was found in the literature that OCT-A can replace the classic dye methods; however, it can complement them. Promoting the possibility that non-invasive OCT-A can substitute the invasive dye methods is deleterious, giving the elusive impression that dye methods are no longer inevitable for evaluating uveitis patients. Nevertheless, OCT-A is a precious tool in uveitis research. [ABSTRACT FROM AUTHOR]

Objectives: The aims of this study were to describe the clinical presentation and treatment modalities of acute retinal necrosis (ARN) and to evaluate complications and clinical outcomes according to the extent of retinal involvement at initial presentation. Materials and Methods: The medical records of 52 patients diagnosed with ARN were reviewed and 48 were included in the study. Patients were categorized into two groups according to the extent of retinitis at presentation: retinal involvement of 1-2 quadrants (Group A) or 3-4 quadrants (Group B). Results: The mean age of the 14 women and 34 men at presentation was 51.3±13.6 years (range: 27-78). There were 40 unilateral and 8 bilateral cases. There were 11 eyes (19.6%) in Group A and 45 eyes (80.4%) in Group B. Eleven patients (22.9%) had a history of herpes simplex virus/varicella-zoster virus infection. One patient in Group A and 11 patients in Group B had received local or systemic corticosteroid therapy without concomitant antiviral treatment before referral. The median follow-up period was 29 months (range: 1-209) in Group A and 8.5 months (range: 0.75-209) in Group B. Mean visual acuity (VA) at presentation was 0.42±0.55 LogMAR (range: 0-2.0) in Group A and 1.28±0.95 LogMAR (range: 0-2.9) in Group B (p<0.05). The presence of endothelial keratic precipitates at presentation was significantly different between two groups (p=0.021). Retinal detachment (RD) occurred in 1 eye (9.1%) in Group A and 30 eyes (66.7%) in Group B (p<0.001). Optic disc pallor was seen in 36.4% (4/11) of eyes in Group A and 71.1% (32/45) of eyes in Group B (p=0.033). Other ocular complications were not significantly different between two groups. Mean final visual acuity was 0.29±0.41 LogMAR in Group A and 1.61±0.90 LogMAR in Group B (p<0.05). Conclusion: The extent of retinal involvement at presentation affects visual outcomes and this shows the importance of early diagnosis and early initiation of antiviral treatment. [ABSTRACT FROM AUTHOR]

Monkeypox is a virus that belongs to the same genus as smallpox and is transmitted through animal-to-human and human-to-human contact. The first case was detected in the Democratic Republic of the Congo in 1970 and subsequent cases have mainly occurred in Western and Central Africa. The discontinuation of smallpox vaccination in 1980 has been attributed to the rise in cases. In 2022, the World Health Organization declared Monkeypox a Public Health Emergency of International Concern. Ocular manifestations of Monkeypox, known as Monkeypox-related ophthalmic disease, have been reported but are rare and typically resolve with conservative management. [Extracted from the article]

To report the results of interferon (IFN) α-2a treatment in patients with cystoid macular edema (CME) secondary to acute retinal necrosis (ARN). We reviewed the records of seven patients (eight eyes) who received IFNα-2a for post-ARN CME. The initial dose of IFNα-2a was 3 MIU/day and it could be tapered down to 3 MIU twice a week. Efficacy was assessed by central macular thickness (CMT) on spectral-domain optical coherence tomography and visual acuity. Age range of seven patients (four men, three women) was 36–74 years. Mean CMT decreased from 477.9 ± 167.5 μm to 367.3 ± 120.5 μm at first week, and vision improved up to five lines in five eyes. CME relapsed after cessation of IFNα-2a in all and improved following reinstitution of treatment. Treatment was discontinued in one patient because of depression. Three patients electively discontinued treatment due to poor tolerability or lack of functional improvement. IFNα-2a is an effective therapeutic option for post-ARN CME, though side effects such as fatigue, elevated liver enzymes, neutropenia, and depression may limit tolerability. Lower initial doses may be a better tolerated. [ABSTRACT FROM AUTHOR]

Purpose: Optical coherence tomography angiography (OCT-A) has been applied to uveitis and intraocular inflammation since its availability after 2014. The imaging of retinal and choroidal vascularization without the use of dyes was a major development and represented a potentially valuable tool in ocular research. In addition to such use, OCT-A is often put forward as being able to potentially replace invasive methods needing dye injection, such as fluorescein angiography (FA) and indocyanine green angiography (ICGA). The aim of this review was to establish whether OCT-A was sufficiently useful in everyday routine clinical practice to monitor disease evolution and to perform treatment adjustments to the extent that it could reliably replace the standard dye methods. Methods: Selective literature review and analysis of own data and experience. Results: OCT-A is a technologically high-grade imaging modality allowing to analyze retinal circulation in inflammatory diseases of the posterior pole with a high sensitivity useful for research purposes. However, there is no evidence that it reaches equal effectiveness in the routine management of posterior uveitis involving the retina. OCT-A is unable to show leakage. In choriocapillaritis involving pre-capillary vessels, it shows capillary drop-out but does not seem to have an advantage over ICGA except that it can be repeated easily, not being invasive, and so allows a closer follow-up. It is, however, less useful in end-choriocapillary non-perfusion, such as in MEWDS. For choroidal stromal inflammation, OCT-A is ill-suited as it only shows inconsistent secondary circulatory changes produced by choroidal foci. OCT-A seems to be useful in the diagnosis and follow-up of inflammatory chorioneovascularisation (iCNV), although dye exams are more precise in showing the activity of the iCNV. Conclusion: In summary, OCT-A is a very sensitive modality for the retinal circulation in uveitis for research purposes; it is sometimes useful for close follow of choriocapillary drop-out but not in end-capillary non-perfusion. Its use for monitoring purposes in stromal choroiditis, however, is questionable. Its claim to possibly replace classical angiographic work-up for the practical management of posterior uveitis is largely overrated. [ABSTRACT FROM AUTHOR]

Sarcoidosis is a common cause of uveitis worldwide, but diagnosing it can be challenging due to overlapping symptoms with other inflammatory diseases. Diagnostic criteria have been developed to standardize the approach to diagnosing sarcoid uveitis. Recent research has focused on the performance of diagnostic tools, presentation patterns, screening for cardiac involvement, and the use of hydroxychloroquine therapy. Other studies have explored the presence of anti-retinal antibodies in sarcoidosis patients, the response to therapy, and the coexistence of other autoimmune conditions. Hydroxychloroquine has shown promise as a treatment option for sarcoid uveitis. [Extracted from the article]

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. Management of immune-mediated choroiditis implies vigorous immunosuppressive therapy given in a prompt and prolonged fashion in most of these entities. [ABSTRACT FROM AUTHOR]

A 20-year-old male Behçet uveitis (BU) patient presented with visual acuities (VAs) of hand movement in OD and counting fingers at 1 m in OS following treatment with corticosteroid monotherapy elsewhere. He had active intraocular inflammation OU along with macular hole and retinal detachment in OS. Infliximab (IFX) was started and vitreoretinal surgery was performed. He had infusion reaction with IFX, hepatotoxicity and depression with interferon, and resistance to adalimumab and tocilizumab therapies. Cytomegalovirus retinitis developed in OD following intravitreal dexamethasone implant and endophthalmitis developed in OS. At the 33rd month of follow-up, the patient was in clinical remission; however, there was persistent angiographic inflammation under certolizumab pegol, cyclosporine, mycophenolate mofetil, and low-dose prednisolone treatment. The left eye was phthisical and VA was 0.4 in OD. Immunomodulatory treatment is given based on the severity of inflammation in BU and needs to be closely monitored for efficacy and adverse effects. [ABSTRACT FROM AUTHOR]

Purpose: To develop an algorithm for the diagnosis of Behçet's disease (BD) uveitis based on ocular findings. Methods: Following an initial survey among uveitis experts, we collected multi-center retrospective data on 211 patients with BD uveitis and 207 patients with other uveitides, and identified ocular findings with a high diagnostic odds ratio (DOR). Subsequently, we collected multi-center prospective data on 127 patients with BD uveitis and 322 controls and developed a diagnostic algorithm using Classification and Regression Tree (CART) analysis and expert opinion. Results: We identified 10 items with DOR >5. The items that provided the highest accuracy in CART analysis included superficial retinal infiltrate, signs of occlusive retinal vasculitis, and diffuse retinal capillary leakage as well as the absence of granulomatous anterior uveitis or choroiditis in patients with vitritis. Conclusion: This study provides a diagnostic tree for BD uveitis that needs to be validated in future studies. [ABSTRACT FROM AUTHOR]

Aims To establish expert recommendations for the management of ocular sarcoidosis (OS). Methods A question-based survey on the management of OS was circulated to international uveitis experts (members of the International Uveitis Study Group and the International Ocular Inflammation Society) electronically. Subsequently, a consensus workshop was conducted at the 7th International Workshop on Ocular Sarcoidosis (IWOS) in June 2019 in Sapporo, Japan as part of the Global Ocular Inflammation Workshops. Statements on the management of OS that were supported by a twothirds majority of 10 international panel members of the workshop, after discussion and voting, were taken as consensus agreement. Results A total of 98 participants from 29 countries responded to the questionnaire survey. The subsequent consensus workshop established recommendations for the management of OS in five sections. The first section concerned evaluation and monitoring of inflammation. The second, third and fourth sections described ocular manifestations that were indications for treatment, and the management of anterior uveitis, intermediate uveitis and posterior uveitis. In the fifth section, the use of systemic corticosteroids and systemic immunosuppressive drugs were detailed. Conclusions Recommendations for management of OS were formulated through an IWOS consensus workshop. [ABSTRACT FROM AUTHOR]

The authors discuss red flags that should have prompted a search for an infectious cause before initiating systemic corticosteroids treatment in this patient, as well as poor prognostic factors in ocular syphilis. 12 Green MB, Agranat JS, Davoudi S, Sanayei N, Ness S. Penicillin responsive presumed seronegative ocular syphilis in a patient with human immunodeficiency virus: a case report and review of the literature. Syphilis has been a constant companion of mankind for many centuries and yet the exact origin of the disease remains unknown.[1] There are currently two main theories regarding the disease's origin.[2] According to the New World theory, also referred to as the Columbian theory, syphilis was endemic to the Caribbean and was probably brought to Europe after the first visit by Columbus in 1492. [Extracted from the article]

Immunomodulatory agents are often used in the systemic treatment of non-infectious uveitis. These drugs consist of corticosteroids, conventional immunosuppressives, and biological agents. As it is known that they suppress the immune system, the most important concern associated with immunomodulatory therapy (IMT) is the increased risk of infection. The World Health Organization declared COVID-19 a pandemic on 11 March 2020. Although severe acute respiratory distress syndrome secondary to SARS-CoV-2 infection may develop in all people, patients who receive IMT may be at higher risk in terms of both the transmission of the infection and more severe disease course. Therefore, guidelines on the management of patients receiving IMT due to uveitis during the pandemic are needed. In this review, we examined the immunomodulatory drugs used in the treatment of uveitis in terms of infectious complications and the data of patients who received IMT during the COVID-19 pandemic and discussed recommendations for the use of these drugs. According to the latest information, patients who receive IMT may continue their treatment as long as there are no disruptions in regular complete blood count (especially white blood cell count >4,000/μL) and liver and kidney function tests. Patients diagnosed with COVID-19 should be managed with a multidisciplinary approach. [ABSTRACT FROM AUTHOR]

Objectives: To analyze and compare sociodemographic features between Behçet uveitis and other non-infectious uveitis. Materials and Methods: The data of adults with non-infectious uveitis in the nationwide uveitis database were analyzed and the sociodemographic features of patients with and without Behçet disease were compared. Results: This study included data of 4,978 eyes of 3,363 patients from 33 centers. The mean age at presentation was 38.7±13.3 (17- 87) years. The mean age was 34.3±10.5 years in the Behçet uveitis group and 41.1±14.0 years in the other non-infectious uveitis group (p<0.001). Male predominance was seen in the Behçet uveitis group (67.7% vs. 32.3%) while female patients were more common in the other non-infectious uveitis group (54.4% vs. 45.6%, p<0.001). Regarding education level, the proportion of patients with low education was higher in the Behçet uveitis group than the other non-infectious uveitis group (49.6% vs. 43.4% in males, p=0.004; 61.5% vs. 59.2% in females, p=0.021). Having a low-income job or being currently unemployed, indicators of poor income, were more frequent in the Behçet uveitis group than in the other non-infectious uveitis group (32.0% vs. 22.8%, p<0.001). In the comparison of places of residence, the proportion of patients who lived in cities with low gross national product was 37.0% in the Behçet uveitis group and 31.1% in the other non-infectious uveitis group (p<0.001). Conclusion: Patients with Behçet disease had lower education level and socioeconomic status than patients with other non-infectious uveitis entities. [ABSTRACT FROM AUTHOR]

The first edition of Ocular Immunology and Inflammation for 2024 has been released, featuring 338 manuscripts that have been processed for publication out of over 700 submissions in 2023. The previous volume of the journal included special topics, such as COVID-19-related articles, infectious uveitis articles, and pediatric uveitis articles. The upcoming volume will focus on "Intermediate Uveitis" as the disease of the year, with experts reviewing epidemiologic, clinical, and imaging features, as well as differential diagnosis and treatment. The editors express gratitude to contributors, reviewing editors, and reviewers for their support and encourage continued submissions to improve knowledge in the field of ocular immunology and inflammation. [Extracted from the article]

Purpose: To provide an overview of the role of multimodality approach to the diagnosis and assessment of uveitic macular edema (UME). Methods: Review of literature. Results: Optical coherence tomography (OCT) has become the gold standard in the detection, quantification, monitoring of treatment, and determination of prognosis in UME. Fluorescein angiography provides essential information on blood-retinal barrier disruption as a result of intraocular inflammation and allows detection of associated retinal ischemia and neovascularization. Dye-less OCT angiography can reveal the presence of various qualitative and quantitative retinal microvascular changes mostly involving the deep capillary plexus. Reading acuity and retinal sensitivity assessed by microperimetry represent sensitive measures for visual dysfunction in UME and may supplement distance visual acuity in the follow-up of patients with UME. Conclusion: Conventional and new imaging modalities and ancillary tests complement each other in the diagnosis, assessment, and monitoring of patients with UME. [ABSTRACT FROM AUTHOR]