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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]

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]

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]

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]

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]

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]