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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]

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]

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]

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]

Diffuse infiltrating retinoblastoma is a rare subtype, occurring in 1% of all patients with retinoblastoma. It usually presents with pseudoinflammatory response in the anterior chamber and the vitreous, masquerading as endophthalmitis or uveitis. This report describes 12-year-old boy with multiple free-floating intraocular pseudocysts as a unique finding in diffuse infiltrating retinoblastoma. These pseudocysts represent necrotic seeds without epithelial lining. Invasive surgical procedures should be avoided in children presenting with atypical, chronic, unilateral intraocular inflammation of unknown cause until retinoblastoma is excluded. [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: 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]

The choroid was poorly accessible to imaging investigation until the last decade of the last century. With the availability of more precise imaging methods such as indocyanine green angiography (ICGA) and, later, optical coherence tomography (OCT), enhanced depth OCT (EDI-OCT), and OCT angiography (OCTA), appraisal of choroidal inflammation has substantially gained in accuracy. This allowed to precisely determine which structures were touched in the different non-infectious choroiditis entities and made it possible to classify this group of diseases, ICGA signs, mainly hypofluorescent lesions, were identified and described. Previous publications have divided angiographic findings into two main sets of signs: (1) irregular "geographic" hypofluorescent areas corresponding to choriocapillaris non-perfusion and (2) round more regular, hypofluorescent dark dots more evenly distributed in the fundus corresponding to more deep choroidal stromal foci. These distinct findings allowed to subdivide and classify choroiditis into choriocapillaritis and stromal choroiditis. Additional signs were identified from EDI-OCT and OCTA examination supporting the classification of choroiditis into choriocapillaritis and stromal choroiditis. Results: Diseases involving principally the choriocapillaris included Multiple Evanescent White Dot Syndrome (MEWDS), Acute Posterior Multifocal Placoid Pigment Epitheliopathy (APMPPE), Idiopathic Multifocal Choroiditis (MFC), and Serpiginous Choroiditis (SC) as well as mixed forms. Diseases primarily involving the choroidal stroma included HLA-A29 Birdshot Retinochoroiditis (BRC), Vogt-Koyanagi-Harada disease (VKH), Sympathetic Ophthalmia (SO), and Sarcoidosis chorioretinitis (SARC). Thanks to new imaging investigations of the choroid, it is now possible to classify and understand the diverse clinicopathological mechanisms in the group of non-infectious choroiditis entities. [ABSTRACT FROM AUTHOR]

Uveitis is occasionally encountered in multiple sclerosis (MS) patients. The objective of this report is to investigate whether uveitis has a prognostic impact on the clinical course of MS. Several clinical and demographic features were compared between 41 MS patients with uveitis and 100 randomly selected MS patients without uveitis. While there were no significant differences by means of gender, age of MS onset, oligoclonal band positivity and disease duration, EDSS and progression index (PI) scores of MS patients with uveitis were significantly lower than those without uveitis (p = 0.004 and <0.001, respectively). Our results suggest that uveitis might be used as a good prognostic factor. [ABSTRACT FROM PUBLISHER]

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