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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]

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

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

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

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

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

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

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

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

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]

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]

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 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]

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]

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: 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]

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]

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]

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]

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).

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]

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]

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 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]

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]

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.

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.

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]

Ocular involvement in Behçet disease (BD) is characterized by recurrent inflammatory attacks and spontaneous resolution of acute inflammatory signs. Both frequency and severity of uveitis attacks determine the magnitude of irreversible damage to intraocular structures and long-term visual prognosis. Recurrent attacks of occlusive retinal vasculitis lead to vision-threatening complications such as cystoid macular edema, retinal neovascularization, optic atrophy and retinal atrophy. This manuscript updates about the role of various drugs in the management of BD, discussing corticosteroids, disease modifying immunosuppressive drugs, and finally biologicals (anti-TNF-blocking agents and alpha interferon) which seem to be superior compared to all other available drugs in preventing loss of vision. Also recent findings from new biologicals will be summarized, and especially the role of these drugs in children will be discussed in detail. The authors suggest that at least moderate to severe retinal involvement should become treated with biologicals whenever available. [ABSTRACT FROM AUTHOR]

Purpose: To describe 3 representative cases of autoimmune retinopathy (AIR). Methods: Clinical records of patients with a diagnosis of AIR were analyzed for demographic data, clinical findings, ancillary and laboratory tests, and treatment employed. Results: Three female patients diagnosed with AIR had bilateral reduction of electroretinogram amplitudes and elevation of visual field threshold within the central 30 degrees of the visual field that was disproportionately more severe than the clinical findings of retinal degeneration. The diagnoses were cancer-associated retinopathy, non-neoplastic AIR, and hereditary retinal dystrophy with secondary inflammation. Optic nerve involvement was also present in all cases. The patient with non-neoplastic AIR was successfully treated with systemic corticosteroids and immunomodulatory agents. Conclusion: High index of suspicion is essential for an early diagnosis of AIR. Visual function and electrophysiological tests should be included in the initial workup of patients who present with suggestive clinical signs and symptoms of AIR. [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]

Juvenile idiopathic arthritis-associated uveitis (JIAU) accounts for a sizable proportion of uveitis cases in children and is an important cause of ocular morbidity in uveitis patients in this age group. The authors present the results of a survey conducted to obtain a better understanding of the current views and practices of ophthalmologists involved in the care of children with JIAU. A detailed questionnaire consisting of 54 questions addressing epidemiology, diagnosis, and therapy of JIAU was distributed to 67 uveitis specialists. The responses from 37 completed questionnaires were tabulated for this report. While the experts often agreed on aspects of the epidemiologic and clinical features of JIAU and its complications, considerable diversity of responses was noted-particularly with regard to practice patterns. Regarding diagnostics and disease monitoring, all experts favored ANA testing, whereas two-thirds also suggested HLA-B27 typing. Laser flare photometry was available to and routinely used by almost one-third of the experts. Optical coherence tomography (OCT) was used by more than half. The survey revealed an overall consensus on therapeutic strategies, including the use of both conventional immunosuppressive and biologic agents. Methotrexate was the initial choice for immunosuppression by most respondents. Most would add an anti-TNF-alpha agent following failure of traditional immunosuppressive therapy, and adalimumab was favored by almost half of the experts. Questions addressing the management of individual situations, such as the treatment of macular edema and perioperative management, revealed considerable differences in therapeutic approaches. The results of this survey support the development of international guidelines for the management of JIAU. [ABSTRACT FROM AUTHOR]

Purpose: To compare Behçet uveitis patients who presented in the 1990s and 2000s.Methods: Records of 170 patients seen in 1990-1994 and 258 patients seen in 2000-2004 were reviewed. Presenting features were analyzed in the whole cohort. Uveitis attacks, vision, complications, and immunomodulatory treatment (IMT) were compared in patients with 3-year follow-up.Results: Posterior segment involvement was less common, visual acuity was better, and more patients received IMT before referral in the 2000s. Follow-up revealed no significant difference in number of uveitis attacks in the first 3 years. Less eyes lost useful vision, no patient became legally blind, and fewer severe ocular complications occurred in the 2000s. IMT was started within 3 months in more patients and interferon alfa-2a was used only in the latter study period.Conclusions: The findings suggest a milder disease at referral, lower rate of severe complications, and a better 3-year visual outcome in patients who presented in the early 2000s. [ABSTRACT FROM AUTHOR]

Any patient with a diagnosis of central serous chorioretinopathy (CSCR) should be questioned to determine any recent corticosteroid use, as the poor visual outcome may be due to unrecognized corticosteroid use. Atypical forms of CSCR may be misdiagnosed as an inflammatory serous retinal detachment, mainly Vogt-Koyanagi-Harada disease. CSCR that develops in association with preexisting uveitis could be a challenging diagnosis as it might be misinterpreted as a worsening of the primary inflammatory condition. Failure to differentiate CSCR from retinal or choroidal inflammatory diseases may result in inappropriate use of corticosteroids, leading to exacerbation of the condition and permanent visual loss. Discontinuation of corticosteroids should be the first step in the treatment of CSCR associated with corticosteroid therapy, and the use of immunosuppressive or immunomodulatory drugs may be required to control systemic or ocular inflammatory disease. The prognosis is usually favorable, but persistent visual loss may occur. [ABSTRACT FROM AUTHOR]

Behçet disease (BD) is a common, complex, and severely debilitating systemic vasculitis. Despite an increasing understanding of BD overall, there remain many critical questions to be addressed in terms of the epidemiology, pathophysiology, and treatment of this condition. The opinions of uveitis experts related to these issues were recently surveyed and are summarized herein. The survey results revealed significant disparities between responders in terms of important concepts regarding the clinical features, diagnosis, and treatment of this important inflammatory eye disease. [ABSTRACT FROM AUTHOR]

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]

Laser flare photometry (LFP) is a method used to detect flare in the anterior chamber (AC). Until now several laser flare photometers have been developed and are in use. LFP is not yet used in all major uveitis centres and, in order to understand this reluctance and know whether use of LFP is justified and should be recommended, an expert meeting was deemed necessary leading to this publication. Besides others results included the following: There is variation between users with regard to how many readings are necessary varying from 3 to 10. It appears appropriate to use at least 6–10 readings. LFP is most useful in patients with anterior uveitis. In patients with posterior and intermediate uveitis, at least 15 photons in the anterior chamber is considered the necessary threshold to be reliable to monitor evolution of inflammation. Factors influencing the measurement of LFP are cataract, corneal opacity, pupil size, intraocular lens and shallow anterior chamber but this is mostly irrelevant in pathological situation. LFP can be used to adjust the management of patients with uveitis, but not necessarily influence treatment. Exceptions may be patients with JIA and Behcet’s Disease. LFP results should always be interpreted in conjunction with the usually clinical observations. There is some evidence that worsening of the flare on two consecutive visits is predictive of a relapse, especially in patients with Behcet’s disease. LFP maybe also useful for predicting rejection of corneal transplant when the cornea is not thickened. [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: To compare the clinical features and outcomes of HLA-B27-positive and HLA-B27-negative acute anterior uveitis (AAU) in a large Turkish patient population.Methods: Forty-four patients with HLA-B27-positive AAU and 65 with HLA-B27-negative AAU were reviewed retrospectively. HLA-B27 typing, gender, laterality, clinical features (fibrinous reaction, hypopyon formation), ocular complications, medical treatment, and final visual acuity were recorded. Statistical analyses included Student's t-test, Fisher's exact test, Yate's corrected Chi2 test, and Mann-Whitney U test. A p-value of <0.05 was considered statistically significant.Results: Forty percent of all patients with AAU were found to be positive for HLA-B27 antigen. HLA-B27-positive AAU was significantly more common in males and HLA-B27-negative AAU in females. Unilateral or bilateral alternating AAU as well as fibrinous reaction and hypopyon formation were more common in HLA-B27-positive patients. Posterior iris synechiae and cataract formation were more common and systemic immunosuppressive agents were exclusively required in patients with HLA-B27-negative AAU. Visual outcome did not differ significantly between the HLA-B27-positive and HLA-B27-negative groups.Conclusions: HLA-B27 association is less common in Turkish patients with AAU than in other populations reported in the literature. Although distinct features of HLA-B27 uveitis were confirmed in our study, visual outcome did not differ from idiopathic AAU. Therefore, we believe that HLA-B27 typing in patients with AAU helps the clinician with the diagnosis. However, it has a limited value in determining the prognosis. [ABSTRACT FROM AUTHOR]

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]