To describe a patient with electronegative ERGs in association with probable TRPM1-related cancer associated retinopathy. A 67-year-old female presented with progressive visual disturbances, including night blindness, cloudy vision, color vison problems, and scotomas. Initial ophthalmic examination revealed mild retinal vasculitis and an epiretinal membrane in one eye. Visual acuity was bilaterally normal. Electroretinography (ERG) showed electronegative waveforms. Subsequent systemic investigation revealed a lung mass, later confirmed to be a small cell carcinoma, with metastases. Western blot analysis and immunohistochemistry were performed. The patient received cisplatin and etoposide chemotherapy, and sub-Tenon triamcinolone injections (STTA). ERGs showed an electronegative waveform with features suggesting pan-retinal loss of On-bipolar cell function. Serum Western blot analysis and immunohistochemistry identified anti-retinal autoantibodies to TRPM1 with binding specificity to the C-terminal region. Further systemic evaluation detected a lung mass, which was later confirmed as small cell lung cancer with metastases. Although initial visual field deterioration was noted, subsequent follow-up showed almost total improvement in visual function, including ERG recovery. Best-corrected visual acuity remained stable at 20/20. Despite the ocular improvements, the patient succumbed to her systemic illness 29 months after the initial visit. Electronegative ERGs are unusual in CAR, which usually affects photoreceptor function. This case, associated with anti-retinal autoantibodies to TRPM1, highlights the need for full systems review in a patient with possible paraneoplastic disease, even in the absence of systemic symptoms.

Importance:Postoperative fungal endophthalmitis following cataract surgery is rare, but 6 cases of fungal endophthalmitis associated with Sarocladium kiliense (formerly Acremonium kiliense) have occurred in a short term. All of these cases developed after conventional cataract surgery. Objective:To report the findings of an outbreak of postoperative fungal endophthalmitis after use of trypan blue solution during cataract surgery. Design, Setting, and Participants:This is a retrospective case series of 6 patients with postoperative fungal endophthalmitis who were referred to a single hospital from 2 clinics in Japan and underwent vitrectomy in 2025. The clinical findings and culture results were evaluated, and samples of the trypan blue solution were cultured. Exposure:Postoperative fungal endophthalmitis following cataract surgery. Main Outcomes and Measures:The treatment and recovery of patients from postoperative fungal endophthalmitis were evaluated based on the clinical and microbiological findings. Results:The age of the 6 patients ranged from 41 to 84 years (mean [SD], 72.7 [14.5] years), and 5 of the 6 patients (83%) were female. The mean (SD) interval from the initial surgery to examination at the hospital was 16 (6.8) days. The mean (SD) preoperative best-corrected visual acuity was 1.77 (0.93) logMAR units (Snellen equivalent, 20/120). Vitrectomy was performed on all eyes, and the vitreous opacities were most prominent in the anterior vitreous. After anterior chamber irrigation around the intraocular lens, vitrectomy with intravitreal antibiotic injection was performed. S kiliense was identified in the aqueous humor of 5 eyes and in the vitreous of 5 eyes. Five eyes were treated with intravitreal voriconazole, and topical voriconazole was administered to all eyes. Systemic treatment with intravenous liposomal amphotericin B or oral voriconazole was also used. Two eyes required reoperations for further removal of the vitreous opacities, including the extraction of the intraocular lens in 1 eye. The vitreous opacities and inflammation improved in all eyes. The infection was controlled in all cases, with a mean (SD) postoperative visual acuity of 0.26 (0.49) logMAR units (Snellen equivalent, 20/30). The same organism was also detected in the trypan blue solution. Conclusions and Relevance:Postoperative endophthalmitis was associated with the presence of S kiliense from the off-label use of contaminated trypan blue solution during cataract surgery.

Purpose To develop imaging and consensus-based guidelines for the application of multimodal imaging in the clinical diagnosis, monitoring and detection of complications in Behçet disease (BD) uveitis. Design International expert consensus agreement using the nominal group technique (NGT) guided by systematic literature review. Participants International uveitis and retina experts participating in the Multimodal Imaging in Uveitis (MUV) taskforce. Methods A committee of experts reviewed the published literature on imaging in BD uveitis, along with representative multimodal imaging datasets of active, resolved and late-stage uveitis in BD. All cases in these datasets met the Standardized Uveitis Nomenclature (SUN) diagnostic criteria. Imaging modalities included color fundus photography (CFP), fundus fluorescein angiography (FFA), optical coherence tomography (OCT), fundus autofluorescence (FAF), indocyanine green angiography (ICGA), and OCT angiography (OCTA). NGT sessions were conducted to define consensus-based key imaging descriptors of active and resolved BD uveitis, along with the complications and sequelae. Main Outcome Measures Identification of reproducible multimodal imaging features of active and resolved BD uveitis. Results The experts agreed that CFP, FFA and OCT are the most relevant imaging modalities in the management of BD uveitis, with particular emphasis on ultra-widefield imaging. Characteristic findings on CFP include vitreous haze, retinal infiltrates, optic nerve head inflammation, and prominent retinal vasculitis with possible occlusion. FFA was deemed critical in assessing vascular, macular and optic nerve head leakage, thereby indicating disease activity. OCT is helpful in detecting and characterizing cystoid macular edema, partial thickness inner retinitis (smudge-sign), subretinal fluid, and overlying vitreous condensation. FFA and OCT assist in demonstrating disease resolution, and detection of late changes such as retinal non-perfusion, neovascularization, epiretinal membrane formation and retinal atrophy. The experts concluded that FAF, ICGA and OCTA have limited role in disease. Based on these findings, consensus-based statements were generated and voted upon by the MUV taskforce. Conclusion Incorporation of consensus-based imaging guidelines by MUV, particularly CFP, FFA and OCT, enhances diagnostic evaluation, improves assessment of disease activity, assessment of treatment response, and detection of complications in BD uveitis. These recommendations provide a structured framework for optimal multimodal imaging use in BD uveitis and aid future refinements of diagnostic criteria.

Methotrexate (MTX) has been widely used for patients with autoimmune diseases as a corticosteroid-sparing agent. We here report an 86-year-old Japanese woman treated with MTX for rheumatoid arthritis who presented with panuveitis along with a yellowish retinal mass. Both Epstein-Barr virus (EBV) and cytomegalovirus (CMV) were detected in aqueous humor and vitreous fluid by quantitative polymerase chain reaction. The retinal mass gradually regressed over eight months after MTX withdrawal. The present case indicates that intraocular MTX-lymphoproliferative disorder (MTX-LPD) should be considered in the differential diagnosis of patients presenting with uveitis who are undertaking long-term MTX therapy. Multiplex PCR along with quantitative PCR for intraocular fluids may be helpful for understanding the pathogenesis of intraocular MTX-LPD.

PurposeTo evaluate the clinical performance of "Direct Strip PCR," a multiplex solid-phase real-time polymerase chain reaction (PCR) kit, for diagnosing infectious uveitis.Study designMulticenter, prospective, diagnostic accuracy studyMethodsWe analyzed 475 ocular-fluid samples (336 aqueous humor and 139 vitreous fluid) from 29 sites in Japan. Direct Strip PCR, an IVD-grade multiplex real-time PCR kit targeting HSV-1, HSV-2, VZV, EBV, CMV, HHV-6, HTLV-1, Toxoplasma gondii, and Treponema pallidum requiring no DNA extraction and incorporating internal controls, was compared with quantitative PCR (qPCR) for concordance and DNA copy-number correlation.ResultsAll 9 of the target pathogens were detected. Direct Strip PCR showed excellent agreement with qPCR, with percent positive agreement (PPA), percent negative agreement (PNA), and percent overall agreement (POA) in aqueous humor of 98.0%, 99.2%, and 98.5%, respectively, and in vitreous fluid of 95.7%, 97.1%, and 96.4%, respectively, indicating high concordance for positive and negative results. All the values exceeded predefined 90% thresholds agreed upon with the Pharmaceuticals and Medical Devices Agency, meeting the required performance criteria. Discordant results were infrequent (10/4275 targets) and involved low-copy samples near the detection limit. The DNA copy numbers correlated strongly between the methods (r = 0.948-0.996). No adverse events were reported.ConclusionDirect Strip PCR demonstrated high concordance with qPCR, reliably detected major pathogens of infectious uveitis, and yielded quantitative results that correlated with the qPCR results, supporting disease monitoring. Its solid-phase, extraction-free, and per-run calibration-free format provides a practical basis for regulatory submissions and global dissemination of multiplex PCR.

PURPOSE:To investigate alterations in circulating microRNAs (miRNAs) in the development of experimental autoimmune uveoretinitis (EAU) in rats. METHODS:Lewis rats were immunized with interphotoreceptor retinoid binding protein (IRBP) peptide (R14) and EAU clinical scores were assessed on day 0 (baseline), and days 7, 14, and 21 after immunization. Total RNA was isolated from serum at the same timepoints and used for microarray analysis. RESULTS:The clinical score of EAU peaked on day 14 and decreased on day 21. Hierarchical cluster analysis and principal component analysis (PCA) of serum miRNA expression displayed distinctly different miRNA profiles between baseline and days 7, 14, and 21 after immunization. Microarray analysis revealed significantly increased expression of 5 (day 7), 9 (day 14), and 10 (day 21) miRNAs, and significantly decreased expression of 19 (day 7), 20 (day 14), and 19 (day 21) miRNAs compared to baseline. Of note, the expression of miRNA-146a-5p, known to be involved in EAU, and miRNA-150-5p was significantly elevated on days 14 and 21. Bioinformatics analysis revealed that mucin type O-glycan biosynthesis and cell adhesion molecules were major pathways affected during the development of EAU. CONCLUSIONS:Hierarchical cluster analysis and PCA showed distinctly different miRNA profiles at baseline versus after IRBP immunization. Upregulation of serum miRNA-146a-5p and miRNA-150-5p was observed in the effector and resolution phases of EAU. Analysis of circulating miRNAs may help to delineate systemic epigenetic changes occurring in the development of EAU, and may lead to new insights in our understanding of human uveitis.

PurposeThis study aimed to investigate demographic features, diagnoses of uveitis (intraocular inflammation), and real-world clinical practice in the use of local and systemic therapies for patients with uveitis in Tokyo, Japan.MethodsClinical records of 1,174 consecutive new patients (480 males, 694 females) referred to the Kyorin Eye Center, Kyorin University Hospital between January 2011 and December 2018 were retrospectively reviewed.ResultsMean age at presentation was 52.6 years (range 4-94 years). By anatomic location, 439 patients (37.4%) had anterior uveitis, 18 (1.5%) had intermediate uveitis, 214 (18.2%) had posterior uveitis and 503 (42.8%) had panuveitis. The 3 most common diagnoses were sarcoidosis (9.1%), Vogt-Koyanagi-Harada (VKH) disease (8.3%), and acute anterior uveitis (5.7%). Compared to our previous study, rates of herpetic anterior uveitis and cytomegalovirus (CMV) retinitis increased while tuberculosis-related uveitis decreased. Unclassified uveitis remained the most common diagnosis (44.9%). Systemic corticosteroids and/or immunomodulatory agents were used in only 18.3% of patients. Immunomodulatory drugs including biologic agents were utilized in 4.9% of patients.ConclusionsThe most common uveitis anatomic type was panuveitis due mainly to high rates of sarcoidosis and VKH disease. Diagnoses of herpetic anterior uveitis and CMV retinitis increased, while tuberculosis-related uveitis decreased. Less than one-fifth of uveitis patients required systemic treatment.

ABSTRACT Objectives This systematic review assessed the efficacy and safety of tumor necrosis factor (TNF) inhibitors in patients with systemic juvenile idiopathic arthritis (JIA). Methods Studies were searched using PubMed, Embase, Cochrane, Ichushi-Web, and clinical trial registries (from 2000 to 2021). The risk of bias was assessed using the Cochrane Risk of Bias version 2 for randomized controlled trials (RCTs) and the manual of Minds for observational studies. Results One RCT and 22 observational studies were included. In the RCT on infliximab, the American College of Rheumatology pediatric (ACR Pedi) 30/50/70 responses at 14 weeks were 63.8%/50.0%/22.4%, with relative risks of 1.30 [95% confidence interval (CI): 0.94–1.79]/1.48 (95% CI: 0.95–2.29)/1.89 (95% CI: 0.81–4.40), respectively. In the observational studies, ACR Pedi 30/50/70 responses for etanercept at 12 months were 76.7%/64.7%/46.4%, respectively. Infliximab treatment caused anaphylaxis in 17% and an infusion reaction in 23% of patients. The incidence of macrophage activation syndrome, serious infection, and malignancy caused by TNF inhibitors was 0–4%. Conclusions Thus, although TNF inhibitors were relatively safe, they were unlikely to be preferentially administered in patients with systemic JIA because of their inadequate efficacy. Further studies, especially well-designed RCTs, are needed to accumulate clinical data.

OBJECTIVES:This systematic review assessed the efficacy and safety of abatacept in patients with systemic juvenile idiopathic arthritis (JIA).METHODS:Studies published between 2000 and 2021 were searched using PubMed, Embase, Cochrane, Ichushi-Web and clinical trial registries. The risk of bias was assessed according to the manual for development clinical practice guidelines by Minds, a project to promote evidence-based medicine in Japan.RESULTS:Seven observational studies were included. American College of Rheumatology pediatric 30/50/70 responses at 3, 6 and 12 months were 64.8%/50.3%/27.9%, 85.7%/71.4%/42.9% and 80.0%/50.0%/40.0%, respectively. Outcomes on systemic symptoms, joint symptoms and activities of daily living were not obtained. No macrophage activation syndrome or infusion reaction occurred. Serious infection occurred in 2.6% of cases.CONCLUSIONS:Abatacept improved the disease activity index. In addition, abatacept was as safe as interleukin-6 (IL -6) and IL-1 inhibitors. However, both the efficacy and safety data in this systematic review should be reviewed with caution because their quality of evidence is low or very low. Further studies are needed to confirm the efficacy and safety of abatacept for systemic JIA, especially its efficacy on joint symptoms.

PurposeTo evaluate 10-year outcome of infliximab (IFX) treatment for uveitis in Behçet disease (BD) patients using a standardized follow-up protocol.DesignRetrospective longitudinal cohort study.Participants140 BD uveitis patients treated with IFX enrolled in our previous study.MethodsMedical records were reviewed for demographic information, duration of IFX treatment, number of ocular attacks before IFX initiation, best corrected visual acuity (VA) at baseline and 1, 2, 3, 4, 5, and 10 years after IFX initiation, uveitis recurrence after IFX initiation and main anatomical site, concomitant therapies, and adverse events (AEs).Main outcome measures10-year IFX continuation rate and change in LogMAR VA.ResultsOf 140 BD patients, 106 (75.7%) continued IFX treatment for 10 years. LogMAR VA improved gradually after initiation of IFX, and the improvement reached statistical significance from 2 years of treatment. Thereafter, significant improvement compared with baseline was maintained until 10 years, despite a slight deterioration of logMAR VA from 5 years. However, eyes with worse baseline decimal VA < 0.1 showed no significant improvement from baseline to 10 years. Uveitis recurred after IFX initiation in 50 patients (recurrence group) and did not recur in 56 (non-recurrence group). Ocular attacks/year before IFX initiation was significantly higher in the recurrence group (2.82 ± 3.81) than in the non-recurrence group (1.84 ± 1.78). In the recurrence group, uveitis recurred within 1 year in 58% and within 2 years in 74%. Seventeen patients (34%) had recurrent anterior uveitis, 17 (34%) had posterior uveitis, and 16 (32%) had panuveitis, with no significant difference in VA outcome. In addition, logMAR VA at 10 years did not differ between the recurrence and non-recurrence groups. AEs occurred among 43 patients (30.7%), and 24 (17.1%) resulted in IFX discontinuation before 10 years.ConclusionsAmong BD patients with uveitis who initiated IFX, approximately 75% continued treatment for 10 years, and their VA improved significantly and was maintained for 10 years. Uveitis recurred in one-half of the patients, but visual acuity did not differ significantly from the patients without recurrence.

“Idiopathic” is the most common category of uveitis, representing cases in which a specific diagnosis has not been established despite work-up. Sarcoidosis is a systemic granulomatous disorder affecting multiple organs including the lungs, skin, kidneys, and eyes. We used microRNA (miRNA) microarrays to investigate serum miRNA profiles of patients with ocular sarcoidosis as diagnosed by specific criteria (diagnosed ocular sarcoidosis), and patients with idiopathic uveitis characterized by ocular manifestations of sarcoidosis (suspected ocular sarcoidosis). Principal component analysis (PCA) and hierarchical clustering showed that serum miRNA profiles of diagnosed ocular sarcoidosis and suspected ocular sarcoidosis were both clearly distinguishable from healthy controls. Furthermore, comparative analysis of the miRNA profiles showed highly similar patterns between diagnosed ocular sarcoidosis and suspected ocular sarcoidosis. Pathway analysis revealed common pathways were involved in the two groups, including those of WNT signaling and TGF-beta signaling. Our study demonstrated a high overlap of differentially expressed serum miRNAs in patients with diagnosed ocular sarcoidosis and suspected ocular sarcoidosis, suggesting that these groups share a similar underlying pathology and may represent possible variants of the disease. Characterization of serum miRNA profiles may provide an opportunity for earlier diagnosis and treatment, and may inform more accurate clinical prognosis in patients with an ocular sarcoidosis phenotype.

Experimental autoimmune uveoretinitis (EAU) is an animal model of non-infectious uveitis and is developed by immunization with retinal antigen, interphotoreceptor retinoid-binding protein (IRBP). Nuclear factor erythroid 2- (NF-E2-) related factor 2 (Nrf2) is responsible for regulating antioxidant and inflammatory responses. In this study, we investigated the role of Nrf2 on the development of EAU. Clinical and pathological examination demonstrated that retinal inflammation was exacerbated in Nrf2 knockout (Nrf2 KO) mice compared to wild type (WT) mice, and the expression of inflammatory cytokines (IFN-γ, IL-6, and IL-17) in the retina was significantly elevated in Nrf2 KO mice. GFAP positive cells (astrocytes) and Iba-1 positive cells (microglia cells) in the retina were more numerous in Nrf2 KO mice compared to WT mice. Furthermore, we examined the suppressive effect of the Nrf2 activator CDDO-Im (2-cyano-3,12 dioxooleana-1,9 dien-28-oyl imidazoline) on the development of EAU. The treatment with CDDO-Im significantly reduced the clinical and pathological score of EAU compared to those of vehicle-treated mice. These findings suggest that Nrf2 plays a regulatory role in the pathogenesis of autoimmune uveoretinitis and the activation of the Nrf2 system may have therapeutic potential for protecting vision from autoimmune neuroinflammation.

Supplementary table 1 Clinical characteristics and treatment of 36 consecutive patients with new-onset acute VKH disease Supplementary Table 2: Relationship between intraocular findings at presentation and subfoveal choroidal thickness at 1 week Please note: The publisher is not responsible for the content or functionality of any supporting information supplied by the authors. Any queries (other than missing content) should be directed to the corresponding author for the article.

Purpose: To identify the clinical characteristics of acute retinal necrosis (ARN) and clarify factors associated with poor visual prognosis.Methods: a nationwide multi-center retrospective chart review study was performed in Japan using data from the medical records of 149 consecutive ARN patients. Demographics, ocular signs, virologic testing of intraocular fluids, and treatment were examined. Factors associated with poor visual prognosis were investigated by regression analysis.Results: At initial presentation, anterior chamber cells or mutton-fat keratic precipitates (97%), unilaterality (93%), and yellow-white retinal lesions (86%) were recognized. In the clinical course, rapid circumferential expansion of retinal lesions (39%), development of retinal break or retinal detachment (55%), and optic atrophy (43%) were recorded. Four variables were identified as associated with poor visual prognosis.Conclusions: The present study identified clinical characteristics and factors associated with poor visual prognosis of ARN.

Background: The aim of this study is to develop an automated evaluation of anterior chamber (AC) cells in uveitis using anterior segment (AS) optical coherence tomography (OCT) images. Methods: We analyzed AS swept-source (SS)-OCT (CASIA 2) images of 31 patients (51 eyes) with uveitis using image analysis software (Python). An automated algorithm was developed to detect cellular spots corresponding to hyper-reflective spots in the AC, and the correlation with Standardization of Uveitis Nomenclature (SUN) grading AC cells score was evaluated. The approximated AC grading value was calculated based on the logarithmic approximation curve between the number of cellular spots and the SUN grading score. Results: Among 51 eyes, cellular spots were automatically segmented in 48 eyes, whereas three eyes (all SUN grading AC cells score: 4+) with severe fibrin formation in the AC were removed by the automated algorithm. The AC cellular spots increased with an increasing SUN grading score (p < 0.001). The 48 eyes were split into training data (26 eyes) and test data (22 eyes). There was a significant correlation between the SUN grading score and the number of cellular spots in 26 eyes (rho: 0.843, p < 0.001). There was a significant correlation between the SUN grading score and the approximated grading value of 22 eyes based on the logarithmic approximation curve (rho: 0.774, p < 0.001). Leave-one-out cross-validation analysis demonstrated a significant correlation between the SUN grading score and the approximated grading value of 48 eyes (rho: 0.748, p < 0.001). Conclusions: This automated anterior AC cell analysis using AS SS-OCT showed a significant correlation with clinical SUN grading scores and provided SUN AC grading values as a continuous variable. Our findings suggest that automated grading of AC cells could improve the accuracy of a quantitative assessment of AC inflammation using AS-OCT images and allow the objective and rapid evaluation of anterior segment inflammation in uveitis. Further investigations on a large scale are required to validate this quantitative measurement of anterior segment inflammation in uveitic eyes.

Supplemental Figure S1. Representative composite color map and binary format images using laser speckle flowgraphy (A) Color fundus photograph of the optic nerve head (ONH). (B) Color map produced using laser speckle flowgraphy (LSFG). A circle was placed around the optic disc to measure mean blur rate (MBR) of the ONH. (C) Composite color map for the optic disc. (D) Binary image for segmentation between vessel areas (white) and tissue areas (black). Supplemental Table S1. Baseline demographics and clinical features Please note: The publisher is not responsible for the content or functionality of any supporting information supplied by the authors. Any queries (other than missing content) should be directed to the corresponding author for the article.

High myopia is a major cause of irreversible visual impairment globally. In the present study, we investigated the microRNA (miRNA) profile in the vitreous of macular hole (MH) and high myopic MH. We performed miRNA analysis using TaqMan® Low Density Arrays (Thermo Fisher Scientific, Waltham, MA, USA) to investigate the circulating vitreous miRNA profile from patients with MH (axial length < 26.5 mm, n = 11) and high myopic MH (axial length ≥ 26.5 mm, n = 11) who underwent pars plana vitrectomy. The vitreous inflammatory cytokine signature was examined in high myopic MH eyes using a multiplex assay. A miRNA-Array analysis revealed that let-7c was significantly up-regulated and miR-200a was significantly down-regulated in high myopic MH eyes compared to those in MH eyes. The bioinformatics analysis for up-regulated miRNA targeted gene identified 23 pathways including mitogen-activated protein kinase (MAPK) and several inflammatory signaling pathways, whereas the bioinformatics analysis for down-regulated miRNA targeted genes showed 32 enriched pathways including phosphoinositide 3-kinase/protein kinase B (PI3K/AKT). The levels of inflammatory cytokines including IP-10, IFN-γ, and MCP-1 were significantly higher in the vitreous of high myopic MH eyes. These results suggest that specific miRNAs expressed in the vitreous may be associated with the pathological condition of high myopic MH and the above mentioned miRNAs may contribute to the development of inflammatory status in the vitreous of high myopic eyes.

Purpose To report on the successful treatment of patients with acute Vogt-Koyanagi-Harada (VKH) disease utilizing the antiviral potential of cyclosporine during the COVID-19 pandemic. Study Design Case series. Methods Clinical records were retrospectively reviewed of 4 patients presenting with new-onset acute VKH disease who elected to receive initial treatment consisting of bilateral sub-Tenon injection of triamcinolone acetonide combined with immediately starting oral cyclosporine without the use of systemic corticosteroids. Results The mean follow-up was 17.0 months. Choroidal thickness decreased to normal with recovery of bilateral best-corrected visual acuity (BCVA) of 1.2 in 3 patients. One elderly patient had decreased BCVA (OD 0.5, OS 0.8) due to cataract progression and mild epiretinal membrane. No recurrences of intraocular were observed in any patients. Mild renal dysfunction developed in 2 elderly patients, but importantly no patients developed COVID-19 disease. Conclusions Oral cyclosporine as the initial systemic treatment of acute VKH disease, in combination with sub-Tenon injection of triamcinolone acetonid, lead to favorable clinical outcomes. Due to the known antiviral properties of cyclosporine, we suggest that this may represent a good treatment strategy for patients during the COVID-19 pandemic.

Retinal vascular leakage is known to be an important biomarker to monitor the disease activity of uveitis. Although fluorescein angiography (FA) is a gold standard for the diagnosis and assessment of the disease activity of uveitis, the evaluation of FA findings, especially retinal vascular leakage, remains subjective and descriptive. In the current study, we developed an automatic segmentation model using a deep learning system, U-Net, and subtraction of the retinal vessel area between early-phase and late-phase FA images for the detection of the retinal vascular leakage area in ultrawide field (UWF) FA images in three patients with Behçet’s Disease and three patients with idiopathic uveitis with retinal vasculitis. This study demonstrated that the automated model for segmentation of the retinal vascular leakage area through the UWF FA images reached 0.434 (precision), 0.529 (recall), and 0.467 (Dice coefficient) without using UWF FA images for training. There was a significant positive correlation between the automated segmented area (pixels) of retinal vascular leakage and the FA vascular leakage score. The mean pixels of automatic segmented vascular leakage in UWF FA images with treatment was significantly reduced compared with before treatment. The automated segmentation of retinal vascular leakage in UWF FA images may be useful for objective and quantitative assessment of disease activity in posterior segment uveitis. Further studies at a larger scale are warranted to improve the performance of this automatic segmentation model to detect retinal vascular leakage.