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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]

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]

Juvenile idiopathic arthritis (JIA) is the most common chronic rheumatic disease and the most common systemic association of uveitis in children. JIA is subdivided into seven clinically more homogeneous subtypes, using the International League of Associations for Rheumatology (ILAR) classification system: systemic arthritis, oligoarthritis, rheumatoid factor (RF)-negative polyarthritis, RF-positive polyarthritis, psoriatic arthritis, enthesitis-related arthritis, and undifferentiated arthritis. Uveitis is very rare in patients with systemic onset JIA and in patients with juvenile-onset rheumatoid arthritis. Patients with early-onset antinuclear antibody (ANA)-positive oligoarticular JIA have the highest risk of developing chronic iridocyclitis. Ocular involvement in enthesitis-related arthritis is characterized by recurrent acute symptomatic iridocyclitis. The diagnosis and differential diagnosis of JIA are based on history, physical examination, and laboratory investigations, including complete blood count, acute-phase reactants, ANA, RF, HLA-B27, and musculoskeletal imaging studies. Recent studies have revealed distinct gene expression profiles that may be useful in the diagnosis, classification, and follow-up of JIA patients. [ABSTRACT FROM AUTHOR]

The diagnosis of Behçet disease is clinical and based on the presence of characteristic ocular and systemic inflammatory manifestations. Patients may present with anterior, posterior, or panuveitis in one or both eyes. The differential diagnosis includes a variety of infectious and noninfectious causes of acute nongranulomatous anterior uveitis, intermediate uveitis, occlusive retinal vasculitis, focal or multifocal retinitis, and necrotizing retinitis. A course characterized by sudden onset with improvement followed by recurrence of inflammatory signs is most typical for Behçet uveitis. [ABSTRACT FROM AUTHOR]

Purpose: To determine the efficacy and safety of different doses of secukinumab, a fully human monoclonal antibody for targeted interleukin-17A blockade, in patients with noninfectious uveitis. Design: Three multicenter, randomized, double-masked, placebo-controlled, dose-ranging phase III studies: SHIELD, INSURE, and ENDURE. Participants: A total of 118 patients with Behçet''s uveitis (SHIELD study); 31 patients with active, noninfectious, non–Behçet''s uveitis (INSURE study); and 125 patients with quiescent, noninfectious, non–Behçet''s uveitis (ENDURE study) were enrolled. Methods: After an initial subcutaneous (s.c.) loading phase in each treatment arm, patients received s.c. maintenance therapy with secukinumab 300 mg every 2 weeks (q2w), secukinumab 300 mg monthly (q4w), or placebo in the SHIELD study; secukinumab 300 mg q2w, secukinumab 300 mg q4w, secukinumab 150 mg q4w, or placebo in the INSURE study; or secukinumab 300 mg q2w, secukinumab 300 mg q4w, secukinumab 150 mg q4w, or placebo in the ENDURE study. Main Outcome Measures: Reduction of uveitis recurrence or vitreous haze score during withdrawal of concomitant immunosuppressive medication (ISM). Other end points included best-corrected visual acuity, ISM use (expressed as a standardized ISM score), and safety outcomes. Results: After completion or early termination of each trial, there were no statistically significant differences in uveitis recurrence between the secukinumab treatment groups and placebo groups in any study. Secukinumab was associated with a significant reduction in mean total post-baseline ISM score (P = 0.019; 300 mg q4w vs. placebo) in the SHIELD study. Likewise, secukinumab was associated with a greater median reduction in ISM score versus placebo in the INSURE study, although no statistical analysis of the difference was conducted because of the small sample size. Overall, there was no loss in visual acuity reported in any treatment group during follow-up in all 3 studies. According to descriptive safety statistics, the frequencies of ocular and nonocular adverse events seemed to be slightly higher among secukinumab groups versus placebo across the 3 studies. Conclusions: The primary efficacy end points of the 3 studies were not met. The secondary efficacy data from these studies suggest a beneficial effect of secukinumab in reducing the use of concomitant ISM. Financial Disclosure(s): Proprietary or commercial disclosure may be found after the references. [Copyright &y& Elsevier]

Anterior uveitis is an inflammation of the uveal tract that involves the iris, anterior part of ciliary body or both. It can be classified as acute or chronic anterior uveitis, according to its clinical course, granulomatous or non-granulomatous anterior uveitis, according to its clinical appearence or infectious or non-infectious anterior uveitis, according to its etiology. It is the commonest form of uveitis and less sight-threatening than posterior segment inflammation, however in some cases it can lead to serious complications such as cataract, glaucoma, and cystoid macular edema. Nevertheless these complications can be prevented if anterior uveitis diagnosed and treated on time. Recognition of ocular findings and diagnostic clues in anterior uveitis is essential in order to identify specific ocular and systemic conditions. [ABSTRACT FROM AUTHOR]

The article evaluates the effectiveness of different imaging techniques in diagnosis and management of Behcet disease. The analysis reveals that fundus photography, fluorescein Angiography (FA) and optical coherence tomography (OCT) are the best methods for evaluating and monitoring Behcet disease patients. The article also describes the images obtained for Behcet disease.

Purpose: To test interobserver agreement in the fluorescein/indocyanine green angiography (FA/ICGA) scoring system for uveitis. Methods: Four observers scored 32 dual FA/ICGAs. Spearman rank correlation was used to analyze correlation between pairs of observers in scores assigned to angiographic signs. Kappa statistics were used to test agreement between pairs of observers in comparative total FA and ICGA scores. Results: The authors found a significant correlation between pairs of observers in scores assigned to all FA signs and all except one ICGA sign. The only discordant sign was early stromal vessel hyperfluoresence on ICGA. There was a moderate to substantial agreement between pairs of observers in comparative FA/ICGA total scores. Conclusions: The level of agreement between uveitis specialists in scoring of dual FA/ICGA indicates that the scoring system tested in this study will be useful for clinical studies of uveitis. Increased experience with this system may further improve its reproducibility. [ABSTRACT FROM AUTHOR]

The article presents information on the prevalence of Behçet disease in developing countries. The disease is described as a multisystem inflammatory disorder, which can be diagnosed when the presence of iritis, oral ulcers, genital ulcers, eye lesions, skin findings, and a positive pathergy test are noted. It also notes the high prevalence of the disease in several countries, including Turkey, Israel and Iran. The demographic features, diagnosis and clinical features of the disease are discussed.

Behçet's disease is a multisystem inflammatory disorder that is most common in countries along the ancient "Silk Road". The eye is the most commonly involved vital organ in Behçet's patients and the typical form of involvement is a relapsing remitting panuveitis and retinal vasculitis. Uveitis is the initial manifestation of the disease in 10-15% of the patients. Anterior uveitis is always nongranulomatous. Diffuse vitritis, retinal infiltrates, sheathing of predominantly retinal veins, and occlusive vasculitis are the typical signs of posterior segment inflammation. Spontaneous resolution of acute inflammatory signs is a diagnostic feature. Fundus fluorescein angiography is the gold standard in monitoring inflammatory activity. Laser flare photometry is a useful noninvasive tool since flare readings correlate with fluorescein angiographic leakage. The most common complications are cataract, maculopathy, and optic atrophy. Male patients have a more severe disease course and worse visual prognosis. Immunomodulatory therapy is indicated in all patients with posterior segment involvement. Corticosteroids combined with azathioprine and/or cyclosporine is used initially. Biologic agents, including interferon alfa and infliximab, are used in resistant cases. Visual prognosis has improved in recent years with an earlier and more aggressive use of immunomodulatory therapy and the use of biologic agents in resistant cases. [ABSTRACT FROM AUTHOR]

Several proteins are investigated as candidate auto-antigens in the pathogenesis of Behçet’s disease (BD). This study aimed to screen the cellular responses to auto-antigen (αB-crystallin, αBC), and microorganism ( Streptococcus sangius KTH-1 BES-1 protein) derived peptides as well as to isopentenyl pyrophosphate (IPP). Peripheral blood mononuclear cells (PBMC) from 22 BD patients and 22 healthy controls (HC) were stimulated in vitro with αBC, BES-1 peptides, IPP and PPD and induced proliferation as well as the secreted interleukin (IL)-12 and interferon (IFN)-γ production levels were measured. We did not observe any significant difference in cellular proliferation between BD patients and HC. Induction of IL-12 secretion with αBC was stronger in BD patients ( P = 0.04) and in the disease subgroup with uveitis ( P = 0.027) compared the HC. When responses to PPD were compared, proliferation of PMBC was lower ( P = 0.03), whereas IL-12 secretion was higher in BD ( P = 0.04) as well as in patients under colchicum treatment ( P = 0.04) and with vascular involvement ( P = 0.006) compared to HC. BES-1373–385 peptide induced also higher IL-12 productions by PBMC of BD patients ( P = 0.017) and of patients with uveitis ( P = 0.013). Finally, IPP stimulated higher IL-12 secretions from PBMC in BD patients ( P = 0.035) and in patients with ( P = 0.02) or without ( P = 0.017) uveitis or arthritis ( P = 0.04), under colchicum treatment ( P = 0.01) or not receiving any immunosuppressive treatment ( P = 0.007) compared to HC. These results suggest a more prominent pro-inflammatory cytokine secretion from PBMC in BD patients compared to HC in response to various antigens including αBC protein, BES-1373–385, IPP and PPD. [ABSTRACT FROM AUTHOR]

The purpose of this study was to investigate the referral patterns, demographic and clinical features, and visual prognosis of patients with sarcoid uveitis seen at 2 tertiary eye care centers in Turkey. A retrospective study was made of 44 patients with uveitis associated with biopsy-confirmed or presumed sarcoidosis. Thirty-four patients (77%) were female and 10 (23%) were male. The mean age at onset of uveitis was 39.8 years. Twenty-seven patients (61%) were referred without a diagnosis of sarcoidosis and 17 (39%) patients were referred with a diagnosis of systemic sarcoidosis. The duration of uveitis prior to referral was 2-15 years in 52% of the patients in the former group. At presentation, 37 patients (84%) had bilateral and 7 patients (16%) had unilateral uveitis, and 17 patients (39%) had only anterior uveitis. The most common ocular findings were granulomatous keratic precipitates, persistent posterior synechiae, and snowball vitreous opacities. Kaplan-Meier survival analysis estimated the risk of recurrence as 30% at 6 months and 61% at 5 years. New complications developed in 18 patients. Potential visual acuity was better than 0.4 in 80% of eyes. The estimated risk of losing potential visual acuity by at least 2 lines was 7% at 6 months and 43% at 5 years. None of the eyes lost useful vision (> 0.1) under the authors' care. The referral patterns suggest underrecognition of sarcoidosis as a cause of uveitis in Turkey. The demographic and clinical features of these patients were mostly similar to those reported from other countries, suggesting that the diagnosis may not be difficult in Turkish patients but requires an increased awareness of the disease in this population. [ABSTRACT FROM AUTHOR]

Purpose: We aimed to describe the demographic and clinical features, ocular manifestations, complications, visual prognosis, and treatment in a large population of Turkish patients with Behçet uveitis. We also aimed to compare visual prognosis between male and female sex and between patients who presented before and after 1990.Design: Observational case series.Methods: A retrospective study of 880 consecutive patients (1,567 eyes) with Behçet uveitis seen at the Uveitis Service, Department of Ophthalmology, Istanbul Faculty of Medicine, Istanbul University, from 1980 to 1998. All patients met the classification criteria of the International Study Group for Behçet''s Disease. Information on the patient''s sex, age at onset of uveitis, ocular features, ocular complications, visual acuity, and systemic treatment was collected.Results: Five hundred ninety-nine patients (68%) were male and 281 (32%) were female. The mean age at onset of uveitis was 28.5 years in male and 30 years in female patients. Ocular involvement was bilateral in 78.1% and unilateral in 21.9% of the patients. Panuveitis was the most common form in both sexes. Fundus lesions as well as sight-threatening complications were more common in males. At the beginning of the follow-up, potential visual acuity was 0.1 or less in 30.9% of eyes in males and 24.2% of eyes in females. Kaplan-Meier survival analysis estimated the risks of losing useful vision (>0.1) at 5 and 10 years for males and females as 21% vs 10% and 30% vs 17%, respectively. Male patients who presented in the 1990s had a significantly lower risk of losing vision compared with male patients who presented in the 1980s.Conclusion: Behçet uveitis starts frequently around the end of the third decade and has a male predominance. The disease is more severe and the risk of losing useful vision is higher in males than in females. However, this risk has been significantly reduced in the 1990s. [Copyright &y& Elsevier]

: PurposeTo describe the demographic and clinical features, complications, treatment, and visual results in patients with childhood-onset Behc¸et uveitis.: DesignObservational case series.: MethodsA retrospective study was made of 36 consecutive patients with Behc¸et disease seen at the Uveitis Service, Department of Ophthalmology, Istanbul Faculty of Medicine, Istanbul University, between January 1975 and January 2002. Inclusion criteria were fulfillment of the classification criteria of the International Study Group for Behc¸et Disease and onset of uveitis at 16 years of age or younger. The medical records of 36 patients with childhood-onset Behc¸et uveitis were reviewed. The main outcome measures were sex, age at onset of uveitis, the initial symptom of Behc¸et disease, clinical ocular features, ocular complications, systemic treatment, complications of treatment, and final visual acuity.: ResultsTwenty-five patients were male, 11 were female. Mean age at onset of uveitis was 13.6 years. The initial symptom was oral ulcer in 63.8% of the patients. The majority of patients (83.3%) had bilateral involvement. Panuveitis was the most common form (86.2%). Retinal vasculitis and retinitis were the most common ocular findings seen in 83.3% and 68.2% of the involved eyes, respectively. Cataract, maculopathy, and optic atrophy were the most common complications seen in 46.9%, 45.4%, and 39.4% of the involved eyes, respectively. Immunosuppressive therapy was administered to 75% of the patients. Response to treatment was variable. The most common complications of systemic treatment were associated with corticosteroid therapy. Final visual acuity was worse than 0.1 in 22.7% of the involved eyes.: ConclusionsChildhood-onset Behc¸et uveitis was more common among males. Bilateral panuveitis with retinal vasculitis and retinitis was the most common form of ocular involvement, similar to the adult patient. The treatment is challenging, as the use oral corticosteroids is associated with significant complications and the response to conventional immunosuppressive therapy is variable. [Copyright &y& Elsevier]

Purpose: Familial occurrence has been reported in approximately 8% of Turkish patients with Behçet's disease. Our aim was to compare the clinical course of ocular Behçet's disease among siblings.Methods: We retrospectively studied five pairs of siblings with ocular involvement of Behçet's disease. No other family member of these siblings had any symptom of Behçet's disease.Results: The siblings comprised three sister-brother pairs, one sister-sister pair, and one brother-brother pair. There was a 5-10-year difference between the age at onset of ocular disease among siblings. Three of the five pairs (two sister-brother, one brother-brother) had a dissimilar course of disease activity. Female siblings had an earlier age at onset and tended to have a worse prognosis than their brothers.Conclusions: It is generally acknowledged that male sex is associated with a worse prognosis. In this study, however, we observed that, among siblings with a dissimilar course, female patients were more severely affected than male patients. Poor ocular prognosis in a patient does not indicate aggressive treatment in his/her sibling. We believe that each sibling should be managed on an individual basis. [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]

Choroidal nevi are the most common benign pigmented lesions of the fundus. Choroidal neovascularization is a rare complication of choroidal nevi. We report herein a young patient managed successfully with intravitreal bevacizumab injections for juxtapapillary choroidal neovascularization secondary to choroidal nevus simulating an inflammatory lesion. [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.