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Purpose: This study aimed to explore the relationship between treatment adherence, visual acuity, quality of life (QoL), depression, and anxiety levels in individuals with Behçet's uveitis (BU). Methods: A total of 55 BU patients and 55 healthy controls completed sociodemographic questionnaires, the Beck Depression Inventory (BDI), Beck Anxiety Inventory (BAI), World Health Organization Quality of Life Questionnaire-BREF version (WHOQOL-BREF), Self-reported Questionnaire About Treatment Compliance, and the Morisky Medication Adherence Scale (MMAS). The scores obtained from these scales were analyzed to assess the relationship between treatment adherence, visual acuity, QoL, depression, and anxiety levels. Results: The results revealed that 36 (65.4%) of the 55 BU patients exhibited depressive symptoms, while 45 (81.8%) of them experienced symptoms of anxiety. Patients with BU demonstrated significantly higher BAI and BDI scores compared to the healthy control group (p < 0.001). Furthermore, the patient group reported lower mean scores across multiple domains of the WHOQOL-BREF questionnaire, including general health, psychological health, social relationships, and environment (p < 0.05). Moreover, a significant association was observed between low treatment adherence and lower values of best-corrected visual acuity (BCVA) (p < 0.05), as well as a higher frequency of uveitis attacks (p = 0.005). Conclusion: Poor treatment adherence in BU patients has a negative effect on final visual acuity outcomes. Moreover, BU patients experience lower QoL and higher rates of depression and anxiety compared to the healthy control group. These findings highlight the importance of addressing treatment adherence and psychological well-being in the management of BU. [ABSTRACT FROM AUTHOR]

Behçet's disease (BD) is a chronic multisystem inflammatory disorder characterized mainly by recurrent oral ulcers, ocular involvement, genital ulcers, and skin lesions, presenting with remissions and exacerbations. Arthritis, gastrointestinal lesions, vasculitis, epididymitis, and central nervous system lesions are also frequently observed disease manifestations in BD patients. This disease is more common in the Asian, Mediterranean, and China regions. The age of onset of the disease is 20 to 40 years. Eye involvement in uveitis is one of the main factors of disability caused by this disease and one of its important complications thatted to blindness. Ocular symptoms of Behcet's disease occur in 40-80% of the etiology of this disease. Ocular Behcet's manifests as uveitis (inflammation of the eye). Uveitis is a prevalent disease that mainly affects young people. It leads to a significant number of visual losses. Uveitis is a common, sight-threatening inflammatory ocular disease and includes multiple heterogeneous clinical entities. The prevalence of various types of uveitis depends upon multiple factors, such as age, sex, race, geographic distribution, environmental influence, genetics, and social habits. Epidemiologic research on uveitis is necessary to understand the etiology and immunopathogenesis of this group of diseases. The prevalence of different types of uveitis depends on several factors such as age, sex, race, geographical distribution, environmental factors, genetics, anatomical location of the inflammatory process (anterior, intermediate, posterior uveitis, pan uveitis), histopathology (granulomatous, non-granulomatous), type of inflammatory process ( acute, chronic, recurrent) and cause (infectious, non-infectious) and social habits. Common sight-threatening ocular complications of Behçet Uveitis (BU) include edema, non-perfusion, neovascularization, and atrophy of the retina and/or optic disc. Among various genetic markers, class I, HLA-B5, and its subclass B51 allele have been reported to have the strongest association with BD, but their roles in idiopathic uveitis are unclear. Many studies have confirmed the association of B*51 with uveitis. In addition to this gene, other genes also play a small role in the occurrence of the disease. BD is associated with the B51 subtype of the HLA-B5 gene, especially the B*5101 allele. The association of HLA-B51 with various manifestations of BD has been reported to be conflicting, partly due to different regional and ethnic backgrounds in various studies. HLA-B27 is the most common type of HLA in patients with uveitis, especially in the anterior uveitis, and it is mostly prognostic because it renders the patients prone to recurrent disease. Genes involved included genes expressing interleukins, chemokines, chemokine receptors, and tumor necrosis factor and genes involved in the complement system. The geographic distribution, the familial forms, and the ethnic origin of patients with Behçet's disease (BD) are many evidence in favor of a genetic background. Although BD usually occurs sporadically, familial aggregation and a higher prevalence in siblings and parents of BD patients have been observed. Although the exact pathogenesis of specific uveitis entities is not clear, accumulating evidence supports a hypothesis that uveitis is caused by an imbalanced immune response induced by an interplay of infectious, traumatic, and environmental factors in individuals with particular genetic backgrounds. In this process, the tissue-resident myeloid cells and the lymphocytes play an important role in both the initiation and the expansion of ocular inflammation.

Abstract Behcet’s disease (BD) is a rare but globally distributed vasculitis that primarily affects populations in the Mediterranean and Asian regions. Behcet’s uveitis (BU) is a common manifestation of BD, occurring in over two-thirds of the patients. BU is characterized by bilateral, chronic, recurrent, non-granulomatous uveitis in association with complications such as retinal ischemia and atrophy, optic atrophy, macular ischemia, macular edema, and further neovascular complications (vitreous hemorrhage, neovascular glaucoma). Although the etiology and pathogenesis of BU remain unclear, numerous studies reveal that genetic factors (such as HLA-B51), dysregulated immune responses of both the innate and adaptive immune systems, infections (such as streptococcus), and environmental factors (such as GDP) are all involved in its development. Innate immunity, including hyperactivity of neutrophils and γδT cells and elevated NK1/NK2 ratios, has been shown to play an essential role in this disease. Adaptive immune system disturbance, including homeostatic perturbations, Th1, Th17 overaction, and Treg cell dysfunction, is thought to be involved in BU pathogenesis. Treatment of BU requires a tailored approach based on the location, severity of inflammation, and systemic manifestations. The therapy aims to achieve rapid inflammation suppression, preservation of vision, and prevention of recurrence. Systemic corticosteroids combined with other immunosuppressive agents have been widely used to treat BU, and beneficial effects are observed in most patients. Recently, biologics have been shown to be effective in treating refractory BU cases. Novel therapeutic targets for treating BU include the LCK gene, Th17/Treg balance, JAK pathway inhibition, and cytokines such as IL-17 and RORγt. This article summarizes the recent studies on BU, especially in terms of pathogenesis, diagnostic criteria and classification, auxiliary examination, and treatment options. A better understanding of the significance of microbiome composition, genetic basis, and persistent immune mechanisms, as well as advancements in identifying new biomarkers and implementing objective quantitative detection of BU, may greatly contribute to improving the adequate management of BU patients.

Behçet's disease (BD) is a chronic multisystem inflammatory disorder characterized mainly by recurrent oral ulcers, ocular involvement, genital ulcers, and skin lesions, presenting with remissions and exacerbations. Arthritis, gastrointestinal lesions, vasculitis, epididymitis, and central nervous system lesions are also frequently observed disease manifestations in BD patients. This disease is more common in the Asian, Mediterranean, and China regions. The age of onset of the disease is 20 to 40 years. Eye involvement in uveitis is one of the main factors of disability caused by this disease and one of its important complications thatted to blindness. Ocular symptoms of Behcet's disease occur in 40-80% of the etiology of this disease. Ocular Behcet's manifests as uveitis (inflammation of the eye). Uveitis is a prevalent disease that mainly affects young people. It leads to a significant number of visual losses. Uveitis is a common, sight-threatening inflammatory ocular disease and includes multiple heterogeneous clinical entities. The prevalence of various types of uveitis depends upon multiple factors, such as age, sex, race, geographic distribution, environmental influence, genetics, and social habits. Epidemiologic research on uveitis is necessary to understand the etiology and immunopathogenesis of this group of diseases. The prevalence of different types of uveitis depends on several factors such as age, sex, race, geographical distribution, environmental factors, genetics, anatomical location of the inflammatory process (anterior, intermediate, posterior uveitis, pan uveitis), histopathology (granulomatous, non-granulomatous), type of inflammatory process ( acute, chronic, recurrent) and cause (infectious, noninfectious) and social habits. Common sight-threatening ocular complications of Behçet Uveitis (BU) include edema, non-perfusion, neovascularization, and atrophy of the retina and/or optic disc. Among various genetic markers, class I, HLA-B5, and its subclass B51 allele have been reported to have the strongest association with BD, but their roles in idiopathic uveitis are unclear. Many studies have confirmed the association of B*51 with uveitis. In addition to this gene, other genes also play a small role in the occurrence of the disease. BD is associated with the B51 subtype of the HLA-B5 gene, especially the B*5101 allele. The association of HLAB51 with various manifestations of BD has been reported to be conflicting, partly due to different regional and ethnic backgrounds in various studies. HLA-B27 is the most common type of HLA in patients with uveitis, especially in the anterior uveitis, and it is mostly prognostic because it renders the patients prone to recurrent disease. Genes involved included genes expressing interleukins, chemokines, chemokine receptors, and tumor necrosis factor and genes involved in the complement system. The geographic distribution, the familial forms, and the ethnic origin of patients with Behçet's disease (BD) are many evidence in favor of a genetic background. Although BD usually occurs sporadically, familial aggregation and a higher prevalence in siblings and parents of BD patients have been observed. Although the exact pathogenesis of specific uveitis entities is not clear, accumulating evidence supports a hypothesis that uveitis is caused by an imbalanced immune response induced by an interplay of infectious, traumatic, and environmental factors in individuals with particular genetic backgrounds. In this process, the tissue-resident myeloid cells and the lymphocytes play an important role in both the initiation and the expansion of ocular inflammation. [ABSTRACT FROM AUTHOR]

PurposeMethodsResultsConclusionsTo examine the demographic characteristics, findings and complication rates in patients with Behçet’s uveitis (BU) and to investigate the effect of early biological therapy on the development of complications.Medical records of 94 patients with BU were retrospectively reviewed. Demographic data, ocular findings and complications at presentation, complications during follow-up, and treatments received during follow-up were analyzed. Patients who were followed for at least 24 months were divided into two groups according to the time of presentation as Group 1 (between 2009 and 2015) and Group 2 (between 2016 and 2021). Complications at the time of presentation and during follow-up, and treatments were compared.We enrolled 94 patients with a male-to-female ratio of 1.94 with a mean age of 30 ± 12 years. Median follow-up was 58.1(12–163) months. There were 35 patients (66 eyes) in Group 1 and 33 patients (61 eyes) in Group 2. At the time of presentation, end-stage disease, cataract, epiretinal membrane, and optic atrophy were significantly more common in Group 1 than in Group 2 (p < 0.05). A significantly higher proportion of eyes in Group 1 developed macular edema, cataract, epiretinal membrane, and macular atrophy during-follow-up (p < 0.05). Median time to initiation of biological treatment was 17.29 months in Group 1 and 3.33 months in Group 2 (p < 0.001). The overall complication rate was significantly lower in Group 2.Prognosis of BU is improved after the introduction of biological treatment. Early use of biological agents in BU is effective in decreasing sight-threatening ocular complications. [ABSTRACT FROM AUTHOR]

4-octyl itaconate (4-OI) is an anti-inflammatory metabolite that activates the nuclear-factor-E2-related factor 2 (NRF2) signaling. In the current work, we investigated whether 4-OI could affect the production of proinflammatory cytokines in Behcet's uveitis (BU) and experimental autoimmune uveitis (EAU). Peripheral blood mononuclear cells (PBMCs) of active BU patients and healthy individuals with in vitro 4-OI treatment were performed to assess the influence of 4-OI on the proinflammatory cytokine production. EAU was induced and used for investigating the influence of 4-OI on the proinflammatory cytokine production in vivo. The flow cytometry, qPCR, and ELISA were performed to detect proinflammatory cytokine expression. NRF2 signaling activation was evaluated by qPCR and western blotting (WB). Splenic lymphocyte transcriptome was performed by RNA sequencing. The NRF2 expression by BU patients-derived PBMCs was lower than that by healthy individuals. After treatment with 4-OI, the proportion of Th17 cells, along with the expression of proinflammatory cytokines (IL-17, TNF-α, MCP-1, and IL-6) by PBMCs, were downregulated, and anti-inflammatory cytokine (IL-10) expression was upregulated, although IFN-γ expression was unaffected. The EAU severity was ameliorated by 4-OI in association with a lower splenic Th1/Th17 cell proportion and increased nuclear NRF2 expression. Additionally, 4-OI downregulated a set of 248 genes, which were enriched in pathways of positive regulation of immune responses. The present study shows an inhibitory effect of 4-OI on the proinflammatory cytokine production in active BU patients and EAU mice, possibly mediated through activating NRF2 signaling. These findings suggest that 4-OI could act as a potential therapeutic drug for the treatment and prevention of BU in the future study. [ABSTRACT FROM AUTHOR]

Behcet's disease (BD) is a rare but globally distributed vasculitis that primarily affects populations in the Mediterranean and Asian regions. Behcet's uveitis (BU) is a common manifestation of BD, occurring in over two-thirds of the patients. BU is characterized by bilateral, chronic, recurrent, non-granulomatous uveitis in association with complications such as retinal ischemia and atrophy, optic atrophy, macular ischemia, macular edema, and further neovascular complications (vitreous hemorrhage, neovascular glaucoma). Although the etiology and pathogenesis of BU remain unclear, numerous studies reveal that genetic factors (such as HLA-B51), dysregulated immune responses of both the innate and adaptive immune systems, infections (such as streptococcus), and environmental factors (such as GDP) are all involved in its development. Innate immunity, including hyperactivity of neutrophils and γδT cells and elevated NK1/NK2 ratios, has been shown to play an essential role in this disease. Adaptive immune system disturbance, including homeostatic perturbations, Th1, Th17 overaction, and Treg cell dysfunction, is thought to be involved in BU pathogenesis. Treatment of BU requires a tailored approach based on the location, severity of inflammation, and systemic manifestations. The therapy aims to achieve rapid inflammation suppression, preservation of vision, and prevention of recurrence. Systemic corticosteroids combined with other immunosuppressive agents have been widely used to treat BU, and beneficial effects are observed in most patients. Recently, biologics have been shown to be effective in treating refractory BU cases. Novel therapeutic targets for treating BU include the LCK gene, Th17/Treg balance, JAK pathway inhibition, and cytokines such as IL-17 and RORγt. This article summarizes the recent studies on BU, especially in terms of pathogenesis, diagnostic criteria and classification, auxiliary examination, and treatment options. A better understanding of the significance of microbiome composition, genetic basis, and persistent immune mechanisms, as well as advancements in identifying new biomarkers and implementing objective quantitative detection of BU, may greatly contribute to improving the adequate management of BU patients. [ABSTRACT FROM AUTHOR]

PurposeMethodsResultsConclusionBehcet’s Disease is a chronic multisystem vasculitis associated with a blinding uveitis. Few comparative studies exist between conventional disease-modifying antirheumatic drugs (DMARDs) and biologic DMARDs in Behcet’s uveitis (BU). We therefore used drug retention time (DRT), an accepted surrogate measure of pharmacological efficacy and tolerability, to compare these treatments in patients with BU.Retrospective chart review of patients who met the revised International Criteria for Behcet’s Disease (ICBD) treated at the Royal Victorian Eye and Ear Hospital, Australia, between 1985–2021. DRT was analysed with Kaplan-Meier plots and defined as total time on drug in the first medication-period for each DMARD in each patient.Forty-eight patients (37 males) with median age of 28.6 years were followed-up for a median of 8.0 years. At initial presentation, half had bilateral disease and median logMAR visual acuity was 0.176 (Snellen 6/9) in 62 uveitic eyes (16 anterior uveitis, 11 intermediate, 2 posterior, and 33 panuveitis). Thirty-three patients met ICBD initially. Prescribed corticosteroid-sparing agents were Cyclosporin (N = 24), Mycophenolate (N = 22), Azathioprine (N = 22), Methotrexate (N = 16), and Adalimumab (N = 15). Median DRT was 14.0, 27.4, 8.3, 24.0, and 52.0 months, respectively. DMARDs were discontinued 116 times and adverse effects (N = 37) were the main reason for cessation. Over time, patients were switched from Cyclosporin to Adalimumab earlier in the disease course due to poorer tolerance of adverse events.Adalimumab’s drug retention time was found to be similar to and possibly better than cDMARDs in patients with BU, who often suffer from vision-threatening disease at first presentation. [ABSTRACT FROM AUTHOR]

Aim: This study aims to evaluate the long‐term efficacy, safety, and cumulative retention rate of antitumor necrosis factor‐alpha (anti‐TNF‐α) therapy for patients with Behcet's uveitis (BU) using meta‐analysis. Methods: We searched the Web of Science and PubMed databases for eligible studies up to December 1, 2022. The quality of each identified study was assessed using the Joanna Briggs Institute's case series literature quality assessment tool. Statistical analysis was conducted using Stata 16.0 software with a random‐effects model. Results: Twelve studies comprising 1156 patients with BU were included in our analysis. We found that 85.0% of patients achieved ocular inflammation remission after receiving anti‐TNF‐α treatment, with a 95% confidence interval (CI) ranging from 78.7% to 90.5%. Additionally, 77.4% (95% CI: 57.5%–92.5%) experienced an improvement in visual acuity (VA). Moreover, the pooled dose reduction of glucocorticoids (GCs) was 11.08 mg (95% CI: −13.34 mg to −8.83 mg). Throughout the follow‐up period, the cumulative retention rate of the medication was 67.3% (95% CI: 53.7%–79.6%). Serious adverse events occurred in 5.8% (95% CI: 3.1%–8.9%) of cases, with the three most common types being severe infusion or injection reactions (2.7%; 95% CI: 0.8%–5.4%), tuberculosis (1.3%; 95% CI: 0.0%–3.9%), and bacterial pneumonia (1.3%; 95% CI: 0.1%–3.4%). Subgroup analysis revealed that ocular inflammation remission rates were 89.3% (95% CI: 81.2%–95.5%) for adalimumab treatment and 83.7% (95% CI: 75.3%–90.8%) for infliximab treatment. The drug retention rate after adalimumab therapy was 70.3% (95% CI: 62.0%–78.0%) compared to 66.4% (95% CI: 48.6%–82.2%) for infliximab treatment. Furthermore, the incidence of severe infusion or injection reactions was 2.2% (95% CI: 0.1%–5.8%) following adalimumab treatment and 3.5% (95% CI: 0.7%–7.7%) following infliximab treatment. Conclusions: Anti‐TNF‐α therapy represents an effective treatment for BU patients with favorable safety profile and high drug retention rate and a potential advantage of adalimumab over infliximab in terms of ocular inflammation remission, drug retention, and the incidence of severe infusion or injection reactions. [ABSTRACT FROM AUTHOR]

Abstract Behçet’s uveitis (BU), a vision-threatening manifestation of Behçet’s disease, poses substantial management challenges due to its chronic, relapsing nature and potential for vision loss. This review explores the role of biologic therapies in the treatment of BU, providing a comprehensive overview of their effectiveness, drawbacks, and future possibilities. Traditionally, management has relied heavily on corticosteroids and conventional immunosuppressants. However, their long-term use is frequently associated with systemic side effects and insufficient control of ocular inflammation. Biologic therapies, particularly TNF-alpha inhibitors like infliximab and adalimumab, have emerged as effective alternatives, offering better disease control and a more favorable safety profile. We critically evaluated these agents, noting their clinical efficacy in reducing inflammatory flares and preserving visual acuity. Despite their benefits, several issues remain. Accessibility, cost, and lack of long-term safety data limit their widespread use. Additionally, individual variability in treatment response necessitates personalized therapeutic strategies. Recent research has shown promise in addressing these challenges, with the emergence of novel biologic agents and personalized medicine approaches. In summary, biologic therapies represent a paradigm shift in BU management, contributing to better patient outcomes. Yet, there are significant challenges to be overcome. As we move forward, continued research, development of novel biologic agents, and a precision medicine approach will shape the future landscape of BU treatment.

Behcet's disease (BD) is a chronic multi-systemic disease characterized by relapsing-remitting oral ulcers, genital ulcers, ocular inflammatory involvements, and numerous other systemic features. Ocular involvements are quite common in BD and may cause severe tissue damage and potentially blindness. Even though the pathogenesis of BD remains ambiguous, growing evidences have shown that genetic factors, environmental triggers and immunological abnormalities play significant roles in its development and progression. Novel biotherapies targeting IFN-g, TNF-a and interleukins have been used in recent years. In this review, we mainly pay attention to the ocular involvement of BD, and discuss the current understanding of mechanisms and advances in therapeutic approaches, especially novel biologics. Finally, we discuss the management in patients with pregnancy. [ABSTRACT FROM AUTHOR]

Behçet's uveitis (BU), a vision-threatening manifestation of Behçet's disease, poses substantial management challenges due to its chronic, relapsing nature and potential for vision loss. This review explores the role of biologic therapies in the treatment of BU, providing a comprehensive overview of their effectiveness, drawbacks, and future possibilities. Traditionally, management has relied heavily on corticosteroids and conventional immunosuppressants. However, their long-term use is frequently associated with systemic side effects and insufficient control of ocular inflammation. Biologic therapies, particularly TNF-alpha inhibitors like infliximab and adalimumab, have emerged as effective alternatives, offering better disease control and a more favorable safety profile. We critically evaluated these agents, noting their clinical efficacy in reducing inflammatory flares and preserving visual acuity. Despite their benefits, several issues remain. Accessibility, cost, and lack of long-term safety data limit their widespread use. Additionally, individual variability in treatment response necessitates personalized therapeutic strategies. Recent research has shown promise in addressing these challenges, with the emergence of novel biologic agents and personalized medicine approaches. In summary, biologic therapies represent a paradigm shift in BU management, contributing to better patient outcomes. Yet, there are significant challenges to be overcome. As we move forward, continued research, development of novel biologic agents, and a precision medicine approach will shape the future landscape of BU treatment. [ABSTRACT FROM AUTHOR]

To investigate the features of macrophage-like cell (MLC) characterized by en face optical coherence tomography (OCT) in Behçet's uveitis (BU). The extent of fluorescein vascular leakage (FVL) was graded on a scale of 0-3 (0=none, 1=mild, 2=moderate, 3=severe) for the optic nerve head (ONH), macula and peripheral retina. The 3μm en face OCT slabs on inner limiting membrane of ONH or macular region was used to visualize the MLCs. The MLC densities of BU group in ONH and macular region were significantly higher than the control group (both p<0.001). The ONH and macular MLC density were significantly higher in eyes with higher FVL grade and they were positively correlated with FVL score (all p<0.001). MLC density was elevated in Behçet's uveitis and it may serve as a noninvasive indicator for the severity of fluorescein leakage and retinal inflammation in Behcet's uveitis. [ABSTRACT FROM AUTHOR]

Purpose. To study the possible impact of human herpes viruses (HHV) reactivation: herpes simplex virus type 1 (HSV 1), type 2 (HSV 2), cytomegalovirus (CMV) and Epstein — Barr virus (EBV) on the clinical course of uveitis in Behcet's disease (BD). Material and methods. Serum samples of 106 BD patients (ave. age 39) with uveitis were examined for the presence of antibodies — serological markers of chronic HHV infection and reactivation. Results. In 65 patients (25 with active uveitis (UA), 40 with uveitis remission (UR)), HHV reactivation was detected (mainly HSV 1, less often HSV 2, CMV and EBV — in individual cases), 41 patients had chronic HHV (17 with UA, 24 with UR). Certain clinical symptoms of uveitis were found to depend on HHV activity. Clinical signs of active uveitis (cells in the vitreous body), as well as severe irreversible changes (social blindness and low vision) were significantly more frequently detected in cases of HHV reactivation (p < 0.05) than in chronic HHV. The data obtained allow us to suggest, with some caution, that a subclinical HHV reactivation which stays after the onset of clinical uveitis remission, is an important factor of postuveal complications that are mainly manifested during remission. These complications include epiretinal fibrosis, pronounced optic nerve atrophy, and vasculitis (arterial and venous occlusion). Conclusion. The remission period in patients with subclinical herpesvirus activity is less favorable than in patients without serological markers of HHV reactivation. HHV reactivation that persists after uveitis activity is stopped can contribute to sudden exacerbations of uveitis.

Abstract Purpose: To investigate the pattern of ocular involvement in Behcet's disease (BD) with predictors of patients' final state of vision. Methods: This historical cohort encompassed the clinical records of 200 patients diagnosed according to the International Criteria for BD (ICBD), over a period of 17 years between 2004 and 2021. Results: The prevalence of Behcet's uveitis (BU) was more common in females and patients in the fourth decade of life. Ninety-five patients (47.5%) had evidence of ocular involvement in the initial ophthalmologic evaluation, and 171 patients (85.5%) manifested evidence of BU during the follow-up visits of which bilateral non-granulomatous panuveitis was the most common anatomical pattern of involvement (32.9%) followed by posterior (27.6%), anterior (26.5%), and intermediate (13.8%) uveitis. The prevalent accompanying signs were oral aphthous (67%), skin lesions (29%), and genital ulcers (19.5%). Cystoid macular edema (CME) was the most frequent ocular complication (62%), followed by cataract (57.5%) and epiretinal membranes (ERM) (36.5%). Univariate analysis showed the following determinants: male gender, younger age at onset, panuveitis, posterior uveitis, retinal vasculitis, and longer duration of uveitis as poorer visual prognostic factors of the disease. Multivariate analysis demonstrated a higher chance of poor visual prognosis of BD in patients with panuveitis, posterior uveitis, retinal vasculitis, and longer duration of uveitis. Conclusion: This cohort study demonstrated an overview on epidemiological patterns of BU along with the visual prognostic factors in Iranian patients.

Purpose: To compare the retinal parameters in Behcet's uveitis (BU) patients with wide-field swept-source optical coherence tomography angiography (SS-OCTA) and find a sensitive OCTA parameter., Methods: Fifty-two eyes from 52 quiescent BU patients and 50 healthy eyes were included. All subjects underwent SS-OCTA examinations with 12 × 12 mm region. Vessel density (VD) and flow area (FA) in nerve fiber layer (NFL), superficial vascular plexus (SVP), intermediate capillary plexus (ICP) and deep capillary plexus (DCP) were analyzed and compared in central, parafoveal, and peripheral regions with diameters of 1, 6 and 12 mm. Receiver operating characteristic curves (ROC), area under the curve (AUC), correlation analysis between OCTA metrics and best-corrected visual acuity (BCVA) were respectively evaluated., Results: BU patients showed significantly lower peripheral VD and FA in NFL (P = 0.019 and 0.002), lower central and parafoveal VD-SVP (P = 0.006 and <0.001), and lower VD-ICP, VD-DCP, FA-SVP, FA-ICP and FA-DCP in all regions (all P < 0.05) as compared to healthy controls. The ROC analysis indicated that the parafoveal, peripheral FA-DCP -1 , and a combination of the two metrics were sensitive parameters for identifying retinal vessel changes in BU (AUC=0.90, 0.90, 0.91, respectively). The parafoveal and peripheral FA-DCP were negatively associated with logMAR BCVA (r=-0.764, P < 0.0001; r=-0.641, P < 0.0001)., Conclusion: The deep retinal layers were apt to be affected in BU patients. The parafoveal and peripheral FA values of DCP may be sensitive parameters for detecting retinal vasculature alterations in BU., Competing Interests: Declaration of competing interest The authors declare no conflict of interests., (Copyright © 2024. Published by Elsevier B.V.)

Purpose: To assess the subfoveal choroidal thickness (SFCT) in patients with Behçet disease (BD) and compare the SFCT in patients with and without ocular BD (OBD) and between patients with active and quiescent phases of the Behçet's posterior uveitis. Method: This prospective cross-sectional study was conducted on patients with BD (n = 51) between October 2016 and October 2018. Complete ocular examinations including slit lamp biomicroscopy and fundus examination with dilated pupils were performed for all patients. The SFCT values were compared between patients with and without OBD. Enhanced depth imaging optical coherence tomography (EDI–OCT) was done to measure the SFCT, and wide field fundus fluorescein angiography (WF-FAG) was performed to evaluate the ocular involvement and determine the active or quiescent phases of the Behçet's posterior uveitis. The correlation between the changes of SFCT and the WF-FAG scores was assessed. Results: One hundred and two eyes of 51 patients with BD, aged 29 to 52 years were studied. Of these, 23 patients were male. The mean age ± standard deviation in patients with OBD and patients without ocular involvement was 38.71 ± 7.8 and 36.22 ± 10.59 years (P = 0.259) respectively. The mean SFCT in patients with OBD was significantly greater than in patients without OBD (364.17 ± 93.34 vs 320.43 ± 56.70 µm; P = 0.008). The difference of mean SFCT between the active compared to quiescent phase was not statistically significant when only WF-FAG criteria were considered for activity (368.12 ± 104.591 vs 354.57 ± 58.701 µm, P = 0.579). However, when the disease activity was considered based on both WF-FAG and ocular exam findings, SFCT in the active group was higher than the inactive group (393.04 ± 94.88 vs 351.65 ± 58.63 µm, P = 0.060). This difference did not reach statistical significance, but it was clinically relevant. Conclusion: Choroidal thickness was significantly increased in BD patients with ocular involvement; therefore, EDI-OCT could be a noninvasive test for evaluation of ocular involvement in patients with BD. The increased SFCT was not an indicative of activity in OBD; however, it could predict possible ocular involvement throughout the disease course.

Purpose: To investigate the pattern of ocular involvement in Behcet's disease (BD) with predictors of patients' final state of vision. Methods: This historical cohort encompassed the clinical records of 200 patients diagnosed according to the International Criteria for BD (ICBD), over a period of 17 years between 2004 and 2021. Results: The prevalence of Behcet's uveitis (BU) was more common in females and patients in the fourth decade of life. Ninety-five patients (47.5%) had evidence of ocular involvement in the initial ophthalmologic evaluation, and 171 patients (85.5%) manifested evidence of BU during the follow-up visits of which bilateral non-granulomatous panuveitis was the most common anatomical pattern of involvement (32.9%) followed by posterior (27.6%), anterior (26.5%), and intermediate (13.8%) uveitis. The prevalent accompanying signs were oral aphthous (67%), skin lesions (29%), and genital ulcers (19.5%). Cystoid macular edema (CME) was the most frequent ocular complication (62%), followed by cataract (57.5%) and epiretinal membranes (ERM) (36.5%). Univariate analysis showed the following determinants: male gender, younger age at onset, panuveitis, posterior uveitis, retinal vasculitis, and longer duration of uveitis as poorer visual prognostic factors of the disease. Multivariate analysis demonstrated a higher chance of poor visual prognosis of BD in patients with panuveitis, posterior uveitis, retinal vasculitis, and longer duration of uveitis. Conclusion: This cohort study demonstrated an overview on epidemiological patterns of BU along with the visual prognostic factors in Iranian patients. [ABSTRACT FROM AUTHOR]

Background. Behçet's disease (BD) is a chronic inflammatory vasculitis affecting multiple organs. Uveitis is frequently seen in patients with BD, especially in Turkish population. Objectives. To investigate vascular endothelial growth factor (VEGF) gene polymorphisms along with the levels of VEGF and VEGF receptors in patients with Behçet's uveitis (BU). Materials and methods. Fifty-five BD-associated uveitis patients and 30 age-and sex-matched controls were included in this case-control study. The genotypes of the single nucleotide poymorphisms (SNPs): rs2010963 (+405G), rs3025039 (+936T) and rs699947 (-2598A) of the VEGF-A gene were determined using real-time polymerase chain reaction (RT-PCR) and serum levels of VEGF and VEGF receptors were measured using enzyme-linked immunosorbent assay (ELISA). Results. No associations of the VEGF gene polymorphisms were observed in BD uveitis patients, but arthritis was present in 53.3% of patients not possessing CT genotype in C3025039→T polymorphism (p = 0.024). Although there were no statistically significant differences in serum VEGF-A, VEGF-C and soluble vascular endothelial growth factor receptor-3 (sVEGFR-3) levels (p < 0.05), serum vascular endothelial growth factor receptor-1 (VEGFR-1) and sVEGFR-3 levels were significantly higher in the BD group (p < 0.001 and p = 0.001, respectively). In addition, VEGF-C/soluble vascular endothelial growth factor receptor-2 (sVEGFR-2) ratio was significantly higher (p < 0.001), while VEGF-A/VEGFR-1 and VEGF-C/sVEGFR-3 ratios were significantly lower (p < 0.001 and p = 0.033, respectively) in BD patients compared to controls. Also, VEGF-C/sVEGFR-3 (p = 0.024, r = 0.37) and VEGF-C/sVEGFR-2 (p = 0.020, r = 0.38) ratios were positively correlated with disease duration. Conclusions. The significant changes in sVEGFR-3 levels and VEGF-C/sVEGFR-3 ratio has shown that lymphangiogenesis processes might take place in the pathogenesis of BD uveitis, and these parameters can be important indicators of evaluation of BD patients with uveitis together with disease duration. [ABSTRACT FROM AUTHOR]

Behçet’s disease is a chronic, multisystem inflammatory disorder characterized by relapsing inflammation. Although its etiopathogenesis has not yet been clarified, both the adaptive and innate immune systems, genetic predisposition, and environmental factors have all been implicated. It is more frequent and more severe in males in the third and fourth decades of life. The eye is the most frequently involved organ in the course of the disease. Ocular involvement (Behçet’s uveitis) is characterized by bilateral recurrent non-granulomatous panuveitis and occlusive retinal vasculitis. Recurrent inflammatory episodes in the posterior segment may lead to permanent vision loss due to irreversible retinal damage and complications such as macular scarring, macular atrophy, and optic atrophy. Early and aggressive immunomodulatory treatment and the use of biologic agents when needed are crucial for preventing recurrences and improving visual prognosis.

To describe two cases of Behçet's retinitis lesions in the macula causing sloughing of retinal tissue forming a full-thickness macular hole. This was a case series study. Case 1 was a 26-year-old presented, known case of Behçet's disease, presented with a large area of retinitis involving the center of the macula with overlying vitritis. One day after the initiation of treatment, vitritis improved, but the retinitis patch sloughed and created a full-thickness macular hole. Case 2 was a 31-year-old male, known case of Behçet's uveitis, who presented vitritis and multiple retinitis patches involving the macula of the left eye. Two weeks after infliximab infusion, the inflammation resolved with medical management, however, the retinitis patch was complicated by a full-thickness macular hole with an inferior rhegmatogenous retinal detachment. Retinitis at the macula can be complicated by a full-thickness macular hole. [ABSTRACT FROM AUTHOR]

Purpose: To investigate the involvement of peripapillary zone vascular abnormalities in Behcet's uveitis (BU) and associated visual dysfunction. We evaluated the retinal and choroidal microvascular features in both macular and peripapillary areas of BU patients to identify vascular abnormalities contributing to reduced best-corrected visual acuity (BCVA) using optical coherence tomography angiography (OCTA).Methods: A prospective, observational study was conducted in 24 eyes of 13 patients with BU and 24 eyes of 15 healthy participants as controls. They received a standard eye examination and were recorded by OCTA measurements of macular and peripapillary areas. The vascular densities of superficial capillary plexus (SCP) and deep capillary plexus (DCP), choroidal flow area, radial peripapillary capillary network (RPCN) density, foveal avascular zone (FAZ) area and perimeter, full retinal thickness (FRT), and peripapillary retinal nerve fiber layer thickness (pRNFLT) were measured.Correlations among microvascular, structural, and functional changes were assessed.Results: Our findings uncovered that the vascular density was significantly reduced in the peripapillary zone of BU eyes compared to healthy eyes, especially in the inferior subfield of the RPCN. The vascular densities of SCP and DCP quadrants within the macular zone had no significant difference between BU and control groups except for DCP density of the nasal parafoveal quadrant. Both FAZ area and perimeter were greater but without statistical significance in the BU group. Compared to healthy eyes, the choriocapillaris flow area was smaller while the FRT and pRNFLT were greater in the BU group. Notably, there was a significant correlation between the reduction in RPCN vascular density and decreased BCVA in BU patients.Conclusion: Based on OCTA, vascular changes associated with BU are more prominent in the peripapillary zone than those in the macular zone. The vascular density of the RPCN could serve as a sensitive indicator to monitoring BU pathogenic progression and treatment response using a non-invasively method of OCTA.

Background: No study has evaluated the effectiveness of Adalimumab (ADA) as first-line in treatment-naïve patients with retinal vasculitis due to Behçet’s Uveitis (BU).Objective: To compare the efficacy of ADA plus conventional therapy and conventional therapy alone as initial treatments in naïve BU patients characterized by retinal vasculitis.Methods: Medical records of BU patients characterized by retinal vasculitis treated with conventional therapy (CT, refers to glucocorticoid and immunosuppressive agents) alone or ADA plus conventional therapy with at least 6 months of follow-up between February 2015 and June 2020 were analyzed. Only patients who were first diagnosed with BU without previous systemic treatment were reviewed. The retinal vasculitis score based on fluorescein angiography (FA), best-corrected visual acuity, glucocorticoid-sparing effect, the number of relapses and ocular complications were evaluated.Results: A total of 45 patients (87 eyes) were included. Twenty-four patients (55.33%) in the CT group were treated with conventional therapy and 21 patients (46.67%) in the ADA group were treated with ADA plus conventional therapy. The inflammatory parameters improved in both groups. FA scores showed significantly greater improvement in ADA group than CT group (p < 0.001). The median number of relapses was significantly lower, and the duration of remission was longer in ADA group than CT group (p < 0.001). At the last visit, a significantly better BCVA improvement (p = 0.024), better inflammation control (anterior chamber inflammation p = 0.017 and vitritis p < 0.001) and lower daily glucocorticoid dosage (p = 0.005) were identified in patients received ADA therapy. In CT group, 1 patient suffered hepatitis B and tuberculosis, 1 had growth retardation, 1 patient had with osteoporosis, then followed by other mild AEs (mostly respiratory upper tract infections); while in ADA group, 1 patient experienced a mild pneumonia (n = 1) while milder AEs were represented mostly by respiratory upper tract infections followed by gastrointestinal discomfort.Conclusion: ADA plus conventional therapy achieved superiority over conventional therapy as initial treatment in naïve BU patients with retinal vasculitis.

Background: No study has evaluated the effectiveness of Adalimumab (ADA) as first-line in treatment-naïve patients with retinal vasculitis due to Behçet's Uveitis (BU). Objective: To compare the efficacy of ADA plus conventional therapy and conventional therapy alone as initial treatments in naïve BU patients characterized by retinal vasculitis. Methods: Medical records of BU patients characterized by retinal vasculitis treated with conventional therapy (CT, refers to glucocorticoid and immunosuppressive agents) alone or ADA plus conventional therapy with at least 6 months of follow-up between February 2015 and June 2020 were analyzed. Only patients who were first diagnosed with BU without previous systemic treatment were reviewed. The retinal vasculitis score based on fluorescein angiography (FA), best-corrected visual acuity, glucocorticoid-sparing effect, the number of relapses and ocular complications were evaluated. Results: A total of 45 patients (87 eyes) were included. Twenty-four patients (55.33%) in the CT group were treated with conventional therapy and 21 patients (46.67%) in the ADA group were treated with ADA plus conventional therapy. The inflammatory parameters improved in both groups. FA scores showed significantly greater improvement in ADA group than CT group (p < 0.001). The median number of relapses was significantly lower, and the duration of remission was longer in ADA group than CT group (p < 0.001). At the last visit, a significantly better BCVA improvement (p = 0.024), better inflammation control (anterior chamber inflammation p = 0.017 and vitritis p < 0.001) and lower daily glucocorticoid dosage (p = 0.005) were identified in patients received ADA therapy. In CT group, 1 patient suffered hepatitis B and tuberculosis, 1 had growth retardation, 1 patient had with osteoporosis, then followed by other mild AEs (mostly respiratory upper tract infections); while in ADA group, 1 patient experienced a mild pneumonia (n = 1) while milder AEs were represented mostly by respiratory upper tract infections followed by gastrointestinal discomfort. Conclusion: ADA plus conventional therapy achieved superiority over conventional therapy as initial treatment in naïve BU patients with retinal vasculitis. [ABSTRACT FROM AUTHOR]

To investigate the effect of plasma-derived exosomal proteins on neutrophil hyperactivation in Behcet's uveitis (BU), we treated neutrophils from healthy controls with plasma-derived exosomes from active BU patients, and determined the level of neutrophil activation by real-time quantitative PCR (RT-qPCR) and cytokine detection assay. The results revealed that exosomes from active BU patients could activate neutrophils as shown by increasing the expression levels of pro-inflammatory cytokines (IL-17 and IL-6), chemokines (IL-8 and MCP-1), and NETs (MPO and ELANE). Label-free quantitative proteomic analysis of plasma-derived exosomes from patients and healthy controls found a remarkably distinct protein profile and identified differentially expressed proteins (DEPs) between the two groups. The results of GO, KEGG, and GSEA enrichment analysis showed that DEPs were enriched in innate immune-mediated and neutrophil hyperactivation-related signaling pathways. The protein-protein interaction (PPI) analysis determined that SHP2 was a downregulated key hub protein in the exosomes of active BU patients. Knockdown of SHP2 in human neutrophil cell lines (NB4 cells) was shown to promote the secretion of pro-inflammatory cytokines, chemokines, and NETs. The converse effects were observed following SHP2 overexpression. In conclusion, we highlighted a pathogenic role of plasma-derived exosomal SHP2 deficiency in facilitating neutrophil activation and suggested that SHP2 might be an immunoprotective factor in BU pathologic process. • By label-free quantitative proteomic analysis, we depicted the plasma exosomal protein profile of Behcet's uveitis (BU). • SHP2 was identified as a downregulated hub protein in BU pathogenesis. • SHP2 deficiency induces neutrophil activation, as shown by higher levels of pro-inflammatory cytokines, chemokines, and NETs. [ABSTRACT FROM AUTHOR]

Vogt-Koyanagi-Harada (VKH) disease and Behcet's uveitis (BU) are the two major vision-threatening uveitis entities. This study performed the first label-free quantitative proteomics on aqueous humor-derived exosomes from 84 patients with VKH or BU to determine their potential roles. Sixty-five differentially expressed proteins (DEPs) and 40 DEPs were detected in the VKH and BU groups, respectively. GO and KEGG analysis showed that DEPs were mainly enriched in the complement-related pathways. The complement C1q subcomponent subunit B (C1QB) was identified as a key exosomal protein, and its expression was significantly increased by western blotting in both diseases. Additionally, the integrated analysis based on the published scRNA-seq data showed that C1QB-containing exosomes were mainly produced by mononuclear macrophages in the anterior segment tissue. Overall, our proteomic profiling highlights that complement-related pathways may be actively involved in the pathogenesis of these two diseases. These pathways may also serve as treatment targets for both diseases. • This study is the first proteomic profiling of the AH-derived exosomes in patients with uveitis. • Differentially expressed proteins were significantly enriched in complement-related pathways in VKH and BU groups. • The upregulated expression of complement-related protein C1QB was found in AH-derived exosomes of VKH and BU patients. [ABSTRACT FROM AUTHOR]

Interleukin (IL)33, a member of the IL1 superfamily, functions as a nuclear factor and mediates biological effects by interacting with the ST2 receptor. Recent studies have described IL33 as an emerging pro-inflammatory cytokine in the immune system, and IL33/ST2 gene polymorphisms have been implicated in the pathogenesis of various immune diseases. However, the underlying mechanisms of IL33/ST2 in Behcet’s disease (BD) remain to be defined. Here, we investigated the association between IL33/ST2 gene polymorphisms and BD in 585 BD uveitis (BDU) patients and 834 healthy controls using Agena MassARRAY iPLEX platform. We found that rs3821204 was associated with the development of BDU. Moreover, the frequency of rs2210463 G allele was lower in patients with genital involvement. Association analysis revealed a much greater genetic difference between complete-type and incomplete-type BD groups, including three SNPs (rs7044343, rs1048274, and rs2210463). Our findings suggest that IL33/ST2 gene polymorphisms are involved in the pathogenesis of BDU. Different genetic backgrounds may exist in complete-type and incomplete-type BD patients.

Purpose: To assess the subfoveal choroidal thickness (SFCT) in patients with Behçet disease (BD) and compare the SFCT in patients with and without ocular BD (OBD) and between patients with active and quiescent phases of the Behçet's posterior uveitis. Method: This prospective cross-sectional study was conducted on patients with BD (n = 51) between October 2016 and October 2018. Complete ocular examinations including slit lamp biomicroscopy and fundus examination with dilated pupils were performed for all patients. The SFCT values were compared between patients with and without OBD. Enhanced depth imaging optical coherence tomography (EDI--OCT) was done to measure the SFCT, and wide field fundus fluorescein angiography (WF-FAG) was performed to evaluate the ocular involvement and determine the active or quiescent phases of the Behçet's posterior uveitis. The correlation between the changes of SFCT and the WF-FAG scores was assessed. Results: One hundred and two eyes of 51 patients with BD, aged 29 to 52 years were studied. Of these, 23 patients were male. The mean age ±standard deviation in patients with OBD and patients without ocular involvement was 38.71 ± 7.8 and 36.22 ± 10.59 years (P = 0.259) respectively. The mean SFCT in patients with OBD was significantly greater than in patients without OBD (364.17 ±93.34 vs 320.43 ±56.70 µm; P = 0.008). The difference of mean SFCT between the active compared to quiescent phase was not statistically significant when only WF-FAG criteria were considered for activity (368.12 ± 104.591 vs 354.57 ± 58.701 µm, P = 0.579). However, when the disease activity was considered based on both WF-FAG and ocular exam findings, SFCT in the active group was higher than the inactive group (393.04 ±94.88 vs 351.65 ±58.63 µm, P = 0.060). This difference did not reach statistical significance, but it was clinically relevant. Conclusion: Choroidal thickness was significantly increased in BD patients with ocular involvement; therefore, EDI-OCT could be a noninvasive test for evaluation of ocular involvement in patients with BD. The increased SFCT was not an indicative of activity in OBD; however, it could predict possible ocular involvement throughout the disease course. [ABSTRACT FROM AUTHOR]

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Interleukin (IL)33, a member of the IL1 superfamily, functions as a nuclear factor and mediates biological effects by interacting with the ST2 receptor. Recent studies have described IL33 as an emerging pro-inflammatory cytokine in the immune system, and IL33 / ST2 gene polymorphisms have been implicated in the pathogenesis of various immune diseases. However, the underlying mechanisms of IL33/ST2 in Behcet's disease (BD) remain to be defined. Here, we investigated the association between IL33/ST2 gene polymorphisms and BD in 585 BD uveitis (BDU) patients and 834 healthy controls using Agena MassARRAY iPLEX platform. We found that rs3821204 was associated with the development of BDU. Moreover, the frequency of rs2210463 G allele was lower in patients with genital involvement. Association analysis revealed a much greater genetic difference between complete-type and incomplete-type BD groups, including three SNPs (rs7044343, rs1048274, and rs2210463). Our findings suggest that IL33/ST2 gene polymorphisms are involved in the pathogenesis of BDU. Different genetic backgrounds may exist in complete-type and incomplete-type BD patients. [ABSTRACT FROM AUTHOR]

Abstract Background To report the switch rate of conventional immunosuppressive (CIS) therapies to the biological agents (BA) in patients with refractory Behcet’s uveitis (BU). Methods In this retrospective study, clinical records were reviewed of 76 patients’ 116 eyes presenting with BU who had been treated with immunosuppressive drug therapy. Mann Whitney U test was used for the intergroup comparisons of parameters without normal distribution as well as calculation of descriptive statistical methods (mean, standard deviation, median, frequency and rate). Wilcoxon Signed Ranks test was used for the intragroup comparisons of parameters without normal distribution. Pearson’s Chi-Square test and Fisher-Freeman-Halton test were used for the comparisons of qualitative data. Results Except for one, all patients were first treated with CIS regimens for BU. Thirty-one patients (41.3%) who were unresponsive to CIS regimens were switched to IFNα2a therapy. After that, eight of these cases were switched to the anti-TNF-α treatments. The presence of initial ocular complications were found to be statistically higher in BA treated patients than the CIS treated cases (p

Behçet's disease is a chronic, multisystem inflammatory disorder characterized by relapsing inflammation. Although its etiopathogenesis has not yet been clarified, both the adaptive and innate immune systems, genetic predisposition, and environmental factors have all been implicated. It is more frequent and more severe in males in the third and fourth decades of life. The eye is the most frequently involved organ in the course of the disease. Ocular involvement (Behçet's uveitis) is characterized by bilateral recurrent non-granulomatous panuveitis and occlusive retinal vasculitis. Recurrent inflammatory episodes in the posterior segment may lead to permanent vision loss due to irreversible retinal damage and complications such as macular scarring, macular atrophy, and optic atrophy. Early and aggressive immunomodulatory treatment and the use of biologic agents when needed are crucial for preventing recurrences and improving visual prognosis. [ABSTRACT FROM AUTHOR]

Objectives: To determine the prevalence of vitreomacular interface (VMI) disorders in patients with Behçet’s uveitis and assess the relationship between VMI disorders and clinical characteristics. Materials and Methods: The macular optical coherence tomography (OCT) images of 160 eyes of 96 patients with Behçet’s uveitis who were being followed in the Uvea-Behçet’s clinic were assessed retrospectively for VMI disorders including posterior vitreous detachment (PVD), epiretinal membrane (ERM), vitreomacular adhesion (VMA), vitreomacular traction (VMT), full-thickness macular hole (FTMH), lamellar hole (LH) and pseudohole. Results: Twenty-four patients (25%) with unilateral and 72 patients with bilateral uveitis were included in the study. Six (30%) of 20 eyes with anterior uveitis and 57 (40.7%) of 140 eyes with posterior uveitis, in total 63 (39.4%) eyes of 160 eyes had at least one VMI disorder. PVD was detected in 4 eyes (2.5%), ERM in 48 eyes (30%), VMA in 12 eyes (7.5%), and LH in 1 eye (0.6%). None of the eyes had VMT, FTMH, or pseudohole. ERM was detected in 13 eyes (8.1%) on fundus examination and 48 (30%) eyes by OCT (p=0.001). VMI was detected in 12 (50%) of 24 eyes with prior intraocular surgery and 51 (37.5%) of 136 eyes without. The mean duration of uveitis was 7.3±5.8 years in patients with VMI disorders and 5.8±7.7 years in patients without (p=0.04). There was no relation between VMI disorders and anatomic location of uveitis, history of past ocular surgery, number of ocular/periocular steroid injections, or visual acuity. Conclusion: VMI disorders are common in patients with Behçet’s uveitis. Their frequency increases with the duration of uveitis. OCT is more sensitive than fundus examination in the detection of VMI disorders.

Purpose: This study investigated the levels of interleukin (IL)-8, IL-10, and vascular endothelial growth factor (VEGF) in the aqueous humor (AqH) of patients with Behçet's uveitis (BU) and Fuchs' uveitis syndrome (FUS) during an inactive period and compared these levels with those in the AqH of noninflammatory healthy control subjects. Methods: This prospective and case–control study included 33 patients (16 patients with BU and 17 patients with FUS) and 35 control subjects. IL-8, IL-10, and VEGF levels in the AqH were quantified by performing sandwich enzyme-linked immunosorbent assay. Kruskal–Wallis test was used to compare the cytokine levels in the different groups, and statistical significance was set at p < 0.05. Results: IL-8 levels were significantly higher in the AqH of patients with BU and FUS than in the AqH of control subjects (p < 0.001 and p < 0.001, respectively). IL-10 levels were significantly lower in the AqH of patients with BU than in the AqH of patients with FUS and of control subjects (p = 0.001 and p < 0.001, respectively). Although VEGF levels were higher in the AqH of patients with FUS than in the AqH of patients with BU and of control subjects, the difference was significant only between patients with FUS and control subjects (p < 0.001). Conclusions: We observed a significant decrease in IL-10 levels in the AqH of patients with BU and a significant increase in VEGF levels in the AqH of patients with FUS compared to controls. IL-8 and VEGF levels showed no significant difference among uveitis patients. [ABSTRACT FROM AUTHOR]

Interferon alpha (IFN-α) is a glycoprotein with antitumor, antiviral, and immunomodulatory activity, used widely in the treatment of viral infections (hepatitis B and C, condylomata acuminata, herpes zoster, etc.), hematological disorders (leukemia, multiple myeloma, T cell lymphoma, and essential thrombocythemia), and solid tumors (clear cell carcinoma in the metastatic stage, melanoma, hepatocellular carcinoma, and cervical neoplasia). Studies have proven the effectiveness of IFN-α in the treatment of ophthalmic disorders involving the anterior segment of the eye (conjunctival papilloma, squamous neoplasia, conjunctival mucosa-associated lymphoid tissue, Mooren's ulcer, and vernal keratoconjunctivitis) and the posterior segment of the eye (serpiginous choroidopathy, posterior uveitis, pseudophakic and diabetic cystoid macular edema, and proliferative diabetic retinopathy). The therapy with IFN-α remains a promising alternative in cases of a failing response to conventional therapy, helping to maintain or improve visual acuity, prevent vision loss, and ameliorate the prognosis of the patient. However, clinicians who decide to use IFN-α in their patients must be aware of general and ophthalmological side effects and inform their patients to undergo a systemic evaluation such as a physical examination, blood and serological tests, and a chest X-ray before the beginning of treatment. This review presents the current knowledge of the use of IFN-α, its efficacy, and properties in ophthalmological diseases, and thus may encourage clinicians to administer this drug as a treatment modality in ophthalmological diseases in the future. [ABSTRACT FROM AUTHOR]

Purpose: To evaluate the ocular surface and meibography of patients with inactive Behçet's uveitis. Methods: Twenty-five right eyes of 25 patients with inactive Behçet's uveitis (Group 1) and 25 right eyes of 25 healthy individuals (Group 2) were enrolled. Detailed eye examination along with Schirmer 1 test, tear film break-up time (t-BUT), ocular surface staining with fluorescein and Oxford scoring, and ocular surface disease index (OSDI) score assessment were performed. Lower and upper eyelid Meibomian glands were examined with infrared filter of slit-lamp biomicroscope (SL-D701 with DC-4 digital camera and BG-5 background illuminator, TOPCON, Tokyo, Japan (from grade 0 (no dropout of Meibomian glands) to grade 3 (gland dropout >2/3 of the total Meibomian glands)). Results: The mean ages were 36.84 ± 7.39 (range, 23–59) and 33.88 ± 8.25 (range, 18–55) in Group 1 and Group 2, respectively (p = 0.547). When compared with Group 2, in Group 1, best-corrected visual acuity (0.28 vs. 0.00 logMAR, p < 0.001), Schirmer 1 test (18.68 vs. 23.69, p = 0.017) and mean tear film break-up time (10.76 vs. 13.36, p = 0.026) were significantly lower; Oxford scale and OSDI scores were higher (p = 0.039 and p < 0.001, respectively). No significant difference in lower, upper and total (upper + lower) meiboscores were detected in between groups (p = 0.062, p = 0.228 and p = 0.152, respectively). Conclusion: Despite the tendency toward dry eye syndrome, Behçet's uveitis does not seem to be associated with quantitative Meibomian gland changes, which is demonstrated by gland drop-out with meibography. [ABSTRACT FROM AUTHOR]

Purpose: To investigate the long-term efficacy and safety of interferon alpha-2a (IFNα-2a) in Chinese patients with Behçet's uveitis (BU) refractory to conventional therapy.Methods: In a prospective observational cohort study, 127 patients were treated with an initial dosage of 3 million units per day in the first three months, followed by gradual tapering of the dose.Results: After 3 months of treatment, IFNα-2a was shown to be effective in 115 cases (91%). At the end of the 1-year follow-up, the frequency of ocular relapses decreased to 1.59 ± 1.68 per year (ranging 0-6) (p < 0.001), as compared to 5.09 ± 2.51 per year (ranging 3-15). Moreover, the frequency of oral ulcer relapses also decreased to 2.49 ± 1.84 per year (ranging 0-6) (p < 0.001), as compared to 8.20 ± 3.72 per year (ranging 2-10). Visual improvement or stability was observed in 32 patients (59%) in these 54 patients. During a mean follow-up of 11 months (range 3-33), the mean final VA (logMAR) had progressed from 1.0 logMAR to 0.8 logMAR in all treated patients.Conclusions: Long-term low dose of IFNα-2a is useful in treating Chinese BU patients who do not respond adequately to conventional therapy. [ABSTRACT FROM AUTHOR]

Purpose: To report on ocular manifestations and visual outcomes of Thai patients with Behçet's Uveitis (BU).Methods: We reviewed medical records of 50 BU patients (31 males and 19 females). Ocular manifestations, treatment modalities, complications, and visual outcomes were registered.Results: Ocular involvement was bilateral in 76% of patients, resulting in 89 affected eyes. Panuveitis and posterior uveitis were the most common types. Retinal vasculitis was noted in majority of affected eyes and specifically arteritis was noticed in 32/57(56%). Most patients received combination therapy of systemic corticosteroids and immunosuppressive agents (azathioprine 72%). At final visit, VA ≤20/200 was observed in 25 affected eyes (28%). Risk factors for poor visual outcome were poor visual acuity at presentation (p < 0.001) and development of optic atrophy (p = 0.01).Conclusions: Typical ocular manifestations of Thai patients with BU consisted of bilateral uveitis affecting posterior eye segment with high rate of complications and frequent visual loss. [ABSTRACT FROM AUTHOR]

Purpose: To assess the long-term efficacy and safety of pegylated interferon alpha-2b (peg-IFN-α-2b) for severe uveitis associated with Behçet's disease.Methods: Peg-IFN-α-2b was administrated at an initial dosage of 0,5-1,5 µg/kg/week. If any complications were observed, the dosage was tapered. Ocular examinations, systemic findings, and treatment compliance of the patients were evaluated.Results: Control of ocular inflammation was achieved in all patients (n = 4). The average increase in visual acuity after drug administration of 0,425 ± 0,125 logMAR was not statistically significant (Wilcoxon Test, p = 0,066) due to small number of cases. Any side-effects with laboratory abnormalities returned to baseline levels with reduction of the doses. Due to less frequent injections, increased satisfaction of the patients was noted.Conclusions: This series highlights the peg-IFN-α-2b administration in Behçet's uveitis. Peg-IFN-α-2b has a potential long-term therapeutic effect for the treatment of severe uveitis. Efficacy with a reduced number of injections improved the quality of Behçet patients' lives. [ABSTRACT FROM AUTHOR]

Behcet's disease (BD) is a chronic multi-systemic disease characterized by relapsing-remitting oral ulcers, genital ulcers, ocular inflammatory involvements, and numerous other systemic features. Ocular involvements are quite common in BD and may cause severe tissue damage and potentially blindness. Even though the pathogenesis of BD remains ambiguous, growing evidences have shown that genetic factors, environmental triggers and immunological abnormalities play significant roles in its development and progression. Novel biotherapies targeting IFN-γ, TNF-α and interleukins have been used in recent years. In this review, we mainly pay attention to the ocular involvement of BD, and discuss the current understanding of mechanisms and advances in therapeutic approaches, especially novel biologics. Finally, we discuss the management in patients with pregnancy., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest. The handling editor WS declared a shared parent affiliation with the author ZL at the time of review., (Copyright © 2023 Lin, Xu, Lin, Xie and Feng.)

Recombinant interferon-α2a (IFNα2a) has been widely used in the treatment of Behcet's uveitis (BU). However, the mechanism underlying its effects remains poorly understood. In this study, we investigated its effect on dendritic cells (DCs) and CD4+ T cells, which are essential for the development of BU. Our results showed that the expression of PDL1 and IRF1 was significantly decreased in DCs from active BU patients, and IFNα2a could significantly upregulate PDL1 expression in an IRF1-dependent manner. IFNα2a-treated DCs induced CD4+ T cells apoptosis and inhibited the Th1/Th17 immune response in association with reduced secretion of IFN-γ and IL-17. We also found that IFNα2a promoted Th1 cell differentiation and IL-10 secretion by CD4+ T cells. Finally, a comparison of patients before and after IFNα2a therapy revealed that the frequencies of Th1/Th17 cells significantly decreased in association with remission of uveitis after IFNα2a therapy. Collectively, these results show that IFNα2a could exert its effects by modulating the function of DCs and CD4+ T cells in BU. [Display omitted] • The expression of PDL1 and IRF1 decreased in DCs of active BU patients. • IFNα2a upregulated PDL1 expression in an IRF1-dependent manner in DCs. • IFNα2a-treated DCs induced CD4+ T cells apoptosis and inhibited Th1/Th17 immune response. [ABSTRACT FROM AUTHOR]

Objectives: To determine the prevalence of vitreomacular interface (VMI) disorders in patients with Behçet's uveitis and assess the relationship between VMI disorders and clinical characteristics. Materials and Methods: The macular optical coherence tomography (OCT) images of 160 eyes of 96 patients with Behçet's uveitis who were being followed in the Uvea-Behçet's clinic were assessed retrospectively for VMI disorders including posterior vitreous detachment (PVD), epiretinal membrane (ERM), vitreomacular adhesion (VMA), vitreomacular traction (VMT), full-thickness macular hole (FTMH), lamellar hole (LH) and pseudohole. Results: Twenty-four patients (25%) with unilateral and 72 patients with bilateral uveitis were included in the study. Six (30%) of 20 eyes with anterior uveitis and 57 (40.7%) of 140 eyes with posterior uveitis, in total 63 (39.4%) eyes of 160 eyes had at least one VMI disorder. PVD was detected in 4 eyes (2.5%), ERM in 48 eyes (30%), VMA in 12 eyes (7.5%), and LH in 1 eye (0.6%). None of the eyes had VMT, FTMH, or pseudohole. ERM was detected in 13 eyes (8.1%) on fundus examination and 48 (30%) eyes by OCT (p=0.001). VMI was detected in 12 (50%) of 24 eyes with prior intraocular surgery and 51 (37.5%) of 136 eyes without. The mean duration of uveitis was 7.3±5.8 years in patients with VMI disorders and 5.8±7.7 years in patients without (p=0.04). There was no relation between VMI disorders and anatomic location of uveitis, history of past ocular surgery, number of ocular/periocular steroid injections, or visual acuity. Conclusion: VMI disorders are common in patients with Behçet's uveitis. Their frequency increases with the duration of uveitis. OCT is more sensitive than fundus examination in the detection of VMI disorders. [ABSTRACT FROM AUTHOR]

Background: To analyze the pattern and causes of visual loss in patients with Behçet's uveitis and to report on the short-term outcome at 6 months and at last follow-up visit. Also, to analyze the pattern of visual acuity changes in eyes with and without macular involvement at the specified time points. Methods: This is a retrospective cohort study of a single-center in an academic practice. Fifty-three patients with Behçet's uveitis evaluated between 2004 and 2014 were included. Data on patients diagnosed with Behçet's uveitis were entered retrospectively into a database and analyzed. Results: Included were 93 eyes with Behçet's uveitis involving the posterior segment. Frequencies of ≤20/50 and of ≤20/200 VA at presentation were 23.7% and 37.6%, respectively. Retinitis, macular inflammatory infiltrate, and dense vitritis were significantly associated with worse vision. Eyes with macular atrophy and macular inflammatory infiltrate sustained the worst logMAR VA at presentation (1.87 and 1.73, respectively) compared to eyes with cystoid macular edema and epiretinal membrane (0.76 and 0.63, respectively). Eyes with no macular involvement had the best VA at presentation. Mean difference in logMAR VA between presentation and the specified time points was greatest for eyes with macular inflammatory infiltrate. Conclusions: Behçet's disease affected mostly young males with a male-to-female ratio of 4.8:1. Panuveitis and posterior uveitis were the predominant forms and they were intrinsically associated with sight-threatening potential and breadth of ocular complications for which aggressive immunosuppressive therapy was essential. [ABSTRACT FROM AUTHOR]