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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]

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]

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]

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]

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]

Systemic vasculitides are a large group of heterogeneous diseases characterized by inflammatory destruction of blood vessels targeting diverse organs and tissues including the eye. As the most vascularized layer of the eye, the choroid is expected to be affected in multiple systemic rheumatologic diseases with vascular involvement. While there are plenty of studies investigating retinal vascular involvement, choroidal vascular involvement in systemic vasculitides has not been investigated in isolation. However, choroidal manifestations including thickness changes, choroidal vasculitis and ischemia may be the earliest diagnostic features of systemic vasculitic diseases. Thus, multimodal imaging of the choroid may help early detection of choroidal involvement which may also have prognostic implications in these life-threatening diseases. This article aimed to review involvement of the choroid in systemic vasculitic diseases. [ABSTRACT FROM AUTHOR]

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]

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]

32 Sanjay S, Handa A, Kawali A, Shetty R, Bhakti Mishra S, Mahendradas P. Scleritis and episcleritis following coronavirus disease (COVID-19) vaccination. Multiple evanescent white dot syndrome following COVID-19 mRNA vaccination. 36 Yoshida N, Tsukada A. A case of neuroretinitis following COVID-19 vaccination. 19 Bellur S, Zeleny A, Patronas M, Jiramongkolchai K, Kodati S. Bilateral acute macular neuroretinopathy after COVID-19 vaccination and infection. [Extracted from the article]

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]

Uveitis is a complex disorder including both infectious and non-infectious etiologies. Clinical diagnosis is a challenge because many diseases share common clinical signs. Laboratory support is crucial for confirming the clinical diagnosis. Laboratory diagnosis includes direct tests and indirect tests. For example smear, culture, and molecular diagnostics demonstrate the pathogens, hence they are direct tests. Immunologic tests employ an antigen to detect presence of antibodies to a pathogen, or an antibody to detect the presence of an antigen, of the pathogen in the specimens. The immunological tests used in laboratories are made by producing artificial antibodies that exactly "match" the pathogen in question. When these antibodies come into contact with a sample they bind to the matching pathogen if found in the sample. Hence they are grouped under indirect evidence. There are several investigations in uveitis to reach the confirmed diagnosis including microbiological, immunological, imaging and molecular diagnostic testing. In this section we will discuss immunological investigations of infectious and non-infectious uveitis. [ABSTRACT FROM AUTHOR]

Purpose: The purpose of this article is to demonstrate the superiority of gevokizumab as compared to placebo, on top of current standard of care, in reducing the risk of Behçet's disease uveitis (BDU) exacerbations.Methods: Randomized, double-masked, placebo-controlled, parallel group, event-driven trial in BDU patients having recently experienced an ocular exacerbation, subsequently undergoing a tapering procedure from high-dose corticosteroids and receiving 60 mg gevokizumab or placebo every 4 weeks subcutaneously (EYEGUARD B-ClinicalTrials.gov NCT 01965145).Results: A total of 83 patients (40 gevokizumab, 43 placebo) were included. Gevokizumab did not significantly affect the risk of occurrence of ocular exacerbations. However, data suggested that gevokizumab could preserve visual acuity, reduce the uveitis severity, decrease the emergence of macular edema, and have a corticosteroid sparing effect. Gevokizumab was well tolerated.Conclusions: While the primary efficacy endpoint was not met with gevokizumab, the control of IL-1β pathway in patients with BDU may still be a relevant target. [ABSTRACT FROM AUTHOR]

Varicella zoster virus (VZV)-induced anterior uveitis (AU) may complicate the course of primary varicella infection typically seen in children. In adults, especially with advanced age, VZV AU is more commonly associated with herpes zoster ophthalmicus (HZO) with or without skin rash affecting the distribution of the ophthalmic nerve due to reactivation of the latent VZV in the trigeminal ganglion. While it is typically a mild self-limiting AU in primary infection, HZO AU is often accompanied by keratitis, may have a chronic recurrent course, and lead to sectoral iris atrophy, pupillary distortion, and ocular hypertension. Diagnosis is often clinical and proven by analysis of aqueous humor for viral genome or antiviral antibodies. Systemic antiviral agents and topical steroids are the mainstay of treatment. Visual prognosis is favorable with timely diagnosis and appropriate treatment. [ABSTRACT FROM AUTHOR]

Uveitis is one of the fields in ophthalmology where a tremendous evolution took place in the past 25 years. Not only did we gain access to more efficient, more targeted, and better tolerated therapies, but also in parallel precise and quantitative measurement methods developed allowing the clinician to evaluate these therapies and adjust therapeutic intervention with a high degree of precision. Objective and quantitative measurement of the global level of intraocular inflammation became possible for most inflammatory diseases with direct or spill-over anterior chamber inflammation, thanks to laser flare photometry. The amount of retinal inflammation could be quantified by using fluorescein angiography to score retinal angiographic signs. Indocyanine green angiography gave imaging insight into the hitherto inaccessible choroidal compartment, rendering possible the quantification of choroiditis by scoring indocyanine green angiographic signs. Optical coherence tomography has enabled measurement and objective monitoring of retinal and choroidal thickness. This multimodal quantitative appraisal of intraocular inflammation represents an exquisite security in monitoring uveitis. What is enigmatic, however, is the slow pace with which these improvements are integrated in some areas. What is even more difficult to understand is the fact that clinical trials to assess new therapeutic agents still mostly rely on subjective parameters such as clinical evaluation of vitreous haze as a main endpoint; whereas a whole array of precise, quantitative, and objective modalities are available for the design of clinical studies. The scope of this work was to review the quantitative investigations that improved the management of uveitis in the past 2-3 decades. [ABSTRACT FROM AUTHOR]

The author reflects on Behcet Uveitis (BU). Topics discussed include Behcet disease (BD) as a systemic disorder; and ocular complications of BU that are edema, non-perfusion, neovascularization, and atrophy of the retina and optic disc. Also being discussed are tumor necrosis factor (TNF) inhibitor or interferon, conventional immunosuppressive therapy, and indocyanine green angiography (ICGA) and fundus autofluroescence (FAF).

Behçet disease is a chronic relapsing multisystem inflammatory disorder. Ocular involvement is characterized by a bilateral recurrent non-granulomatous panuveitis and retinal vasculitis. Posterior segment findings vary during the course of the disease, in parallel with the relapsing and remitting intraocular inflammation. Structural alterations occur with increased disease duration. Fluorescein angiography is the gold standard in revealing the extent and severity as well as the leaky and/or occlusive nature of retinal vasculitis. Multimodal imaging using color fundus photography, fluorescein angiography, and optical coherence tomography is essential in visualizing diagnostic features, detecting structural changes, and monitoring disease activity and response to treatment in patients with Behçet uveitis. [ABSTRACT FROM PUBLISHER]

Purpose: To evaluate agreement and estimate sensitivity and specificity of uveitis specialists' interpretation of ocular photographs in diagnosing Behçet uveitis. Methods: Fourteen Turkish uveitis specialists, masked to demographic and clinical features of patients, independently labeled ocular photographs (29 Behçet/30 other diagnoses) as 'Behçet uveitis' or 'non-Behçet.' Level of agreement was evaluated using kappa statistics. Photographs were categorized based on ocular signs captured and performance of observers. Results: Exact agreement with the correct diagnosis was 56-81%. Seven reviewers correctly labeled more than 70% of photographs. Interobserver agreement among those 7 reviewers revealed moderate ( κ = 0.41-0.60) or substantial ( κ = 0.61-0.80) agreement in 76% of pairs. Smooth layered hypopyon, superficial retinal infiltrate with retinal hemorrhages, and branch retinal vein occlusion with vitreous haze were correctly recognized as Behçet uveitis by majority of reviewers. Conclusions: There are ocular signs of Behçet disease that can be considered diagnostic even in the absence of any other clinical information. [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]

Background: Multiple evanescent white dot syndrome (MEWDS) is a rare inflammatory eye condition affecting the outer retina as a consequence of choriocapillaris non perfusion. The pathophysiology of MEWDS will be discussed based clinical appraisal and on multimodal imaging appraisal. Methods: Narrative review and perspective opinion. Results: Literature review results helped us to put forward (1) the specific symptomatology (decreased/blurred vision, photopsia, subjective scotomas), (2) the ill-asserted character of clinical findings (foveal granularity, white dots in fundoscopy), (3) and the crucial importance of multimodal imaging with the diagnostic triad of ICGA hypofluorescent areas, BL-FAF hyperautofluorescent areas and loss/damage of IS/OS-ellipsoid zone on SD-OCT that characterise the disease and can practically help the clinician to diagnose MEWDS. A comprehensive alternative perspective of the disease was formulated. Conclusions: The bulk of evidence that we are presenting in this review, thanks to new performing non-invasive and invasive imaging modalities, is sufficiently compelling to consider MEWDS as a primary choriocapillaritis/inflammatory choriocapillaropathy. Multimodal imaging allows the clinician to diagnose MEWDS with a high level of certainty and ensures a precise follow-up. [ABSTRACT FROM AUTHOR]

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]

Introduction: The EyeCOPE study characterized noninfectious intermediate posterior, or panuveitis (NIIPPU) before biologic agents were widely available. Methods: This retrospective, observational study included adults with NIIPPU attending a routine ophthalmological visit. Data were collected from the study visit and medical records. Results: Of 565 patients, 58.8% were female, and the mean age was 41.3 years; 33.8% had idiopathic uveitis and 45.8% had panuveitis. The median time from symptom onset to diagnosis and treatment was 27.0 and 30.5 days, respectively. Patients received immunosuppressants and systemic/local corticosteroids. Most patients experienced substantial decline in ocular function (mean best corrected visual acuity, 0.4 logMAR). Mean total work productivity impairment among employed patients was 31.0%. Most patients reported ocular complications (70.8%) such as vision loss and cataracts. Conclusions: Despite treatment, most patients with NIIPPU experienced a decline in ocular function and ocular complications. There is an unmet need for additional NIIPPU treatment, such as targeted monoclonal antibodies. [ABSTRACT FROM AUTHOR]

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]

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]

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]

Purpose: To report the results of tocilizumab (TCZ) treatment in patients with Behçet uveitis (BU) who had failed conventional, interferon alpha, and anti-Tumor necrosis factor-alpha therapy.Methods: We reviewed the records of five patients with BU treated with monthly infusions of TCZ 8 mg/kg. Outcome measures were visual acuity, anterior chamber cells, laser flare meter (LFM) readings, vitreous haze, central macular thickness (CMT), and fluorescein angiography (FA) score.Results: The clinical inactivity of BU and 20/50 or better vision were achieved in three female and two male patients treated with TCZ for 5-19 months. The mean LFM reading was reduced from 15.4 ± 2.7 to 5.0 ± 0.9 ph/ms; the mean CMT from 324.7 ± 36.6 µm to 280.2 ± 34.1 µm; and the mean FA score from 20.6 ± 5.4 to 9.3 ± 4.5 µm at the last visit. The only side effect was a slight elevation of the total cholesterol level in one patient.Conclusions: Tocilizumab may be a safe and effective therapeutic option for refractory BU. [ABSTRACT FROM AUTHOR]

An introduction is presented in which the editors discuss various reports in the issue on topics including the rate of rhegmatogenous retinal detachment (RRD), orbital apex syndrome (OAS), and presentation of uveitis in patients with Herpes Zoster Ophthalmicus (HZO).

The viral causes of anterior uveitis (AU) emerged with the use of novel molecular diagnostic tests and serologic tests adapted for small volumes (Goldmann-Witmer Coefficient). The viral causes of AU may be underestimated, and some of the presumed idiopathic AU cases will probably be proven to be of viral origin in the coming years. So far, a viral origin of AU was suspected in patients who presented with unilateral hypertensive AU. It is not clear which clinical presentations should raise a suspicion of viral etiology. There is an overlap in the clinical manifestations of AU caused by viruses and other non-viral forms of AU. A viral cause of AU should be suspected in patients with unilateral AU, exhibiting small or medium sized KPs, some form of iris atrophy, high IOP and early development of a cataract and the definitive diagnosis can be proven by aqueous humor analysis. [ABSTRACT FROM AUTHOR]

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]

An introduction is presented in which editor discusses articles including overview of pathogenesis of familial Mediterranean fever, occurrence of ocular complications in juvenile idiopathic arthritis patients and a case study of a 62-year-old woman with tubulointerstitial nephritis with uveitis.

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]

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]

An introduction to articles published within the issue is presented, including two articles on the pathogenesis, clinical presentation, diagnosis and management of Vogt-Koyanagi-Harada (VKH) disease, another on a study which examined the serum interleukin (IL)-25 levels in patients in the acute uveitic phase and those with chronic/recurrent inflammation, and a case of a woman diagnosed with Holmes-Adie syndrome.

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 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]

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. [ABSTRACT FROM AUTHOR]

6 Xia A, Dietrich-Ntoukas T, Pleyer U. Effect of anti-TNF treatment on Mooren's ulcer: a case series and review of the literature. The authors cited other reports of the use of TNF inhibitors[[8], [10]] and of rituximab[12] to treat refractory MU and suggested that such agents be considered prior to surgery in those patients who are incompletely responsive to more conventional therapies. Mooren's ulcer (MU)[[1], [3]] is an uncommon cause of corneal ulceration observed most often in Asia and Africa. [Extracted from the article]

Purpose: To analyze the role of polymerase chain reaction (PCR) of ocular fluids in management of tubercular (TB) anterior, intermediate, posterior, and panuveitis. Methods: In Collaborative Ocular Tuberculosis Study (COTS)-1 (25 centers, n = 962), patients with TB-related uveitis were included. 59 patients undergoing PCR of intraocular fluids (18 females; 53 Asian Indians) were included. Results: 59 (6.13%) of COTS-1 underwent PCR analysis. PCR was positive for Mycobacterium TB in 33 patients (23 males; all Asian Indians). 26 patients were PCR negative (18 males). Eight patients with negative PCR had systemic TB. Anti-TB therapy was given in 18 negative and 31 PCR cases. At 1-year follow-up, five patients with positive PCR (15.15%) and three with negative PCR (11.54%) had persistence/worsening of inflammation. Conclusions: Data from COTS-1 suggest that PCR is not commonly done for diagnosing intraocular TB and positive/negative results may not influence management or treatment outcomes in the real world scenario. [ABSTRACT FROM AUTHOR]

Purpose: Relapsing ocular inflammation occurs in about 70% of patients with Behçet's disease (BD) and can lead to permanent loss of vision. Neovascularization of the optic disc (NVD) or elsewhere in the retina (NVE) is a relatively uncommon but severe complication that lacks standardized treatment.Methods: We report on the therapeutic use of anti-TNF monoclonal antibodies for BD-associated NVD and NVE in one pediatric patient (subcutaneous adalimumab) and one young man (intravenous infliximab). Also, we review the previously published experience on biologic therapeutic options, namely anti-TNF agents and interferon-alpha in a total of three and eight patients, respectively.Results: A fast-onset therapeutic effect was observed in both patients leading to complete regression of neovascularizations.Conclusions: Both options may lead to regression of neovascularization, thus preventing loss of vision, but comparative studies need to determine the optimal treatment for this sight-threatening complication of BD. [ABSTRACT FROM AUTHOR]

Purpose: To screen for psychological disorders in patients with active uveitis.Methods: Patients were screened for depression (BDI-II), state anxiety (STAI-I), VR-QOL (NEI-VFQ-25), and HR-QOL (SF-36). Association of depression and anxiety with sociodemographic and clinical parameters and with VR-QOL and HR-QOL were analyzed. Multivariate linear regression models were constructed for NEI-VFQ-25 and SF-36 subscales.Results: Of 99 patients, 37.3% screened positive for depression and 52.5% for anxiety. Depressed patients had lower visual acuity in the better seeing eye (p = 0.013) and more frequently panuveitis (p = 0.018). Anxious patients were younger (p = 0.009), had earlier onset of uveitis (p = 0.015), and had more frequently panuveitis (p = 0.016). Bivariate comparisons showed significant associations between psychological disorders and VR-QOL and HR-QOL. Significant bivariate associations were mostly lost in multivariate analyses for anxiety, but were preserved for depression.Conclusions: A positive screening test for depression and anxiety is common in patients with uveitis. Low vision and panuveitis are associated with depression. Depression is associated with impairment of VR-QOL and HR-QOL. [ABSTRACT FROM AUTHOR]

Purpose: To describe the demographic and clinical profiles of uveitis patients seen at secondary and tertiary care centers in Turkey.Methods: A nationwide web-based registry of patients with uveitis was initiated in November 2008. We analyzed data from a single baseline registry-enrollment visit.Results: In 33 centers, 6967 eyes of 4863 consecutive patients were registered. The mean age at presentation was 36.6 ± 15.7 (1-92) years; 51.3% were male. Behçet disease was the leading diagnosis (24.9%), followed by ankylosing spondylitis and/or HLA-B27-associated anterior uveitis (9.7%), toxoplasmosis (7.1%), Fuchs uveitis (6.3%), and presumed herpetic anterior uveitis (6.0%). Visual acuity was 0.1 or worse in 22% of eyes. The most common ocular complications were posterior synechiae (16.2%) and cataract formation (16.2%).Conclusions: Behçet disease is still the most common non-infectious etiology in Turkish uveitis patients, while ocular toxoplasmosis and herpetic anterior uveitis are the most common infectious uveitic entities. [ABSTRACT FROM AUTHOR]

Purpose: To report the efficacy and safety of interferon (IFN) α-2a in patients with cystoid macular edema (CME) associated with presumed ocular tuberculosis (TB).Methods: We reviewed the clinical records of 5 patients with presumed ocular TB who had been treated with IFN α-2a for recurrent CME during or after completion of anti-tubercular therapy. IFN α-2a was administered at an initial dose of 3 million IU per day and then tapered after the initial response. Treatment efficacy was assessed by central macular thickness (CMT) measurement using spectral-domain optical coherence tomography and visual acuity.Results: Three patients were men, and 2 were women. Patients were aged between 38 and 66 years. Mean CMT was 483 ± 178.6 μm at baseline, 302.3 ± 56 μm at 1 week, 312.3 ± 49.5 μm at 1 month, and 286.2 ± 31.9 μm at 3 months. Mean LogMAR visual acuity was 0.6 ± 0.4 at baseline, 0.4 ± 0.3 μm at 1 week, 0.3 ± 0.3 at 1 month, and 0.3 ± 0.3 at 3 months. The treatment was interrupted for 10 days because of neutropenia after 2 weeks in 1 patient and discontinued in another after 10 days because of intolerance. Total treatment duration was 3-24 months in the remaining 4 patients.Conclusion: The present small case series suggests that IFN α-2a may be an effective and safe therapeutic option for CME that is associated with presumed ocular TB. [ABSTRACT FROM AUTHOR]

Aim To outline recommendations from an expert committee on the assessment and investigation of patients with severe inflammatory eye disease commencing immunosuppressive and/or biologic therapy. Method The approach to assessment is based on the clinical experience of an expert committee and a review of the literature with regard to corticosteroids, immunosuppressive drug and biologic therapy and other adjunct therapy in the management of patients with severe sight-threatening inflammatory eye disease. Conclusion We recommend a careful assessment and consultative approach by ophthalmologists or physicians experienced in the use of immunosuppressive agents for all patients commencing immunosuppressive and/or biologic therapy for sight threatening inflammatory eye disease with the aim of preventing infection, cardiovascular, metabolic and bone disease and reducing iatrogenic side effects. [ABSTRACT FROM AUTHOR]

Purpose: To evaluate the safety and efficacy of gevokizumab for the treatment of Behçet's disease uveitis in a prospective, open-label, randomized phase 2 trial.Methods: Behçet's disease patients with new acute ocular exacerbation or at risk of exacerbation received 30 or 60 mg gevokizumab every 4 weeks intravenously or subcutaneously, on top of a stable regimen of immunosuppressives and corticosteroids (≤20 mg/day equivalent prednisolone). Patients withdrew in cases of ocular exacerbation.Results: A total of 21 patients were included (17 acute and 4 at-risk; mean duration of uveitis 45.6 ± 37.4 months). There were no serious adverse events related to gevokizumab. Recorded adverse events were mostly associated with exacerbation of uveitis or its complications. Response was evaluated for 14 acute patients and all showed rapid control of acute ocular exacerbation, mostly within 1 week, without any increase in corticosteroid dosage.Conclusions: Gevokizumab was well tolerated and rapidly controlled acute ocular exacerbations of Behçet's disease uveitis without the need for high-dose corticosteroid. [ABSTRACT FROM AUTHOR]

During the last decades the efficacy of biologic agents, mainly of anti-TNFs, in controlling the activity of serious manifestations of Behcet's Disease (BD) has been established. On the other hand, the clinical heterogeneity of BD has precluded the validation of a widely-accepted composite index for disease assessment and for target disease-state definitions, such as low disease activity and remission, and the testing of their implementation in clinical practice. Therefore, in contrast to other systemic rheumatic diseases, a treat-to-target strategy has not yet been developed in BD. There are several challenges towards this approach, including standardization of outcome measures for assessing the disease activity in each-affected organ and construction of a composite disease activity index. The challenges for the development of a treat-to-target strategy and possible solutions are discussed in this position paper, which stemmed from a round table discussion that took place in the 19th International Conference on BD. • Expression and severity of the clinical features of BD varies. Therefore measuring overall disease activityis challenging • Efforts are ongoing to determine the best instruments to assess disease activity in BD • Given the emerging therapies in BD, definition of therapeutic targets and development of treat-to-target strategy is needed. [ABSTRACT FROM AUTHOR]

Purpose: To describe fulminant toxoplasma retinochoroiditis induced by corticosteroid monotherapy.Methods: Clinical records of nine patients were reviewed.Results: All patients (five female, four male; aged 15-64 years) had been misdiagnosed as unilateral non-infectious uveitis and given systemic and/or local corticosteroid injections elsewhere. Mean disease duration before referral was 105.6 ± 71 (45-240) days. Visual acuity at presentation was <20/200 in six eyes. Average lesion size was 6.6 disc areas in eight eyes and all four quadrants were involved in one. Toxoplasma DNA was detected in eight tested eyes. Mean duration of anti-toxoplasmic therapy was 92.5 ± 37.1 days. Three eyes developed rhegmatogenous retinal detachment. Four patients underwent pars plana vitrectomy. Final visual acuity was <20/200 in five eyes.Conclusions: Iatrogenic immunosuppression due to initial misdiagnosis may lead to an aggressive course and serious complications of ocular toxoplasmosis, a potentially self-limiting infection. [ABSTRACT FROM AUTHOR]