A Proposal of a New Tool for the Assessment of Damage in Behçet Syndrome Uveitis: Cerrahpasa Ocular Damage Grading System.

INTRODUCTION/OBJECTIVE: There is currently no tool available to assess the severity of damage in uveitis due to Behçet's syndrome (BS). In this preliminary study, we developed a new grading system to evaluate ocular damage and assessed it in a prospective cohort. METHODS: A specialist in BS uveitis (YO) developed a grading system for ocular damage with five grades based on the extent of damage in the posterior segment. YO trained a senior and general ophthalmologist with sample fundus images. BS patients who had undergone color fundus photography during their routine visits in attack-free periods were included in the study. The color fundus photos of this prospective cohort were evaluated blindly by YO and his trainees using the new grading tool. Inter and intra-observer agreement between the graders were assessed by Cohen's kappa analysis. The evaluation of YO was considered as the gold standard. RESULTS: One hundred eighty-five eyes of 108 (29 F/79 M) patients with BS uveitis were graded for damage by two investigators. Their mean age was 38,58 years and their median ocular disease duration was 13 years. The gold standard and the two investigators exhibited substantial concordance with the ocular damage grading system. The inter- and intra-observer agreement were also almost perfect. CONCLUSION: The newly developed ocular damage grading system enables the standardization of damage severity in BS uveitis. It is imperative to conduct internal and external validations across diverse cohorts. Furthermore, future studies should investigate its correlation with other multimodal imaging methods such as fluorescein angiography and optical coherence tomography.

Mycophenolate mofetil may be an alternative for maintenance therapy of Behçet syndrome uveitis: a single-center retrospective analysis.

Experience with mycophenolate in uveitis due to Behçet syndrome (BS) is limited. Twelve patients with panuveitis or posterior uveitis who were started mycophenolate were included. Data on demographic characteristics, therapies, ocular attacks, and adverse events were extracted from patient charts. Seven patients with BS uveitis were prescribed mycophenolate for remission induction, of which 6 were refractory/intolerant to conventional immunosuppressives. Mycophenolate was combined with anti-TNFs in 3 patients, resulting in no further ocular attacks. Mycophenolate had to be stopped in the fourth patient due to adverse events. The remaining 3 patients continued to have ocular attacks and were switched to other agents without any drop in visual acuity. Among the 5 patients who were prescribed mycophenolate for maintenance, 2 were relapse free, but 3 experienced ocular attacks. One patient had an exacerbation of mucocutaneous lesions, and 2 experienced adverse events. Mycophenolate monotherapy may not be adequate for remission induction of refractory BS uveitis, but it can be a safe and effective alternative when combined with a biologic agent. It may also be an option for maintenance therapy.

Pediatric Uveitis in Turkey: The National Registry Report II.

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

Emergence of new manifestations during infliximab treatment in Behçet's syndrome.

OBJECTIVES: Infliximab (IFX) is increasingly being used for the treatment of severe manifestations of Behçet's syndrome (BS). However, emergence of new manifestations has also been occasionally reported during IFX treatment. We aimed to assess the frequency of new manifestations in our BS patients treated with IFX. METHODS: A chart review was conducted to identify all BS patients treated with IFX in our clinic between 2004 and 2020. Demographic data, indications for IFX initiation, concomitant treatments and outcomes were recorded. A new manifestation was defined as the emergence of a new organ involvement or mucocutaneous manifestation developing for the first time during IFX treatment or within 12 weeks after the last infusion of IFX. RESULTS: Among our 282 patients who used IFX, 19 (7%) patients had developed a total of 23 new manifestations during a mean follow-up of 20.0 (15.3) months. Patients with vascular involvement were more likely to develop a new manifestation (12/19, 63%). Initial manifestations that required IFX were in remission at the time of new manifestation in 14/19 patients. IFX treatment was intensified (n = 6) and/or glucocorticoids, immunosuppressives or colchicine was added to IFX (n = 21). IFX was switched to another agent for the remaining manifestations (n = 8). These treatment modifications led to remission in 17/19 patients. CONCLUSION: New manifestations developed during IFX treatment in 7% of our patients with BS. They could be managed by intensifying IFX treatment or adding other agents in the majority of these manifestations.

Comparison of Sociodemographic Features Between Behçet Uveitis and Other Non-infectious Uveitis

Objectives: To analyze and compare sociodemographic features between Behçet uveitis and other non-infectious uveitis. Materials and Methods: The data of adults with non-infectious uveitis in the nationwide uveitis database were analyzed and the sociodemographic features of patients with and without Behçet disease were compared. Results: This study included data of 4,978 eyes of 3,363 patients from 33 centers. The mean age at presentation was 38.7±13.3 (17-87) years. The mean age was 34.3±10.5 years in the Behçet uveitis group and 41.1±14.0 years in the other non-infectious uveitis group (p<0.001). Male predominance was seen in the Behçet uveitis group (67.7% vs. 32.3%) while female patients were more common in the other non-infectious uveitis group (54.4% vs. 45.6%, p<0.001). Regarding education level, the proportion of patients with low education was higher in the Behçet uveitis group than the other non-infectious uveitis group (49.6% vs. 43.4% in males, p=0.004; 61.5% vs. 59.2% in females, p=0.021). Having a low-income job or being currently unemployed, indicators of poor income, were more frequent in the Behçet uveitis group than in the other non-infectious uveitis group (32.0% vs. 22.8%, p<0.001). In the comparison of places of residence, the proportion of patients who lived in cities with low gross national product was 37.0% in the Behçet uveitis group and 31.1% in the other non-infectious uveitis group (p<0.001). Conclusion: Patients with Behçet disease had lower education level and socioeconomic status than patients with other non-infectious uveitis entities.

An Algorithm for the Diagnosis of Behçet Disease Uveitis in Adults.

Purpose: To develop an algorithm for the diagnosis of Behçet's disease (BD) uveitis based on ocular findings.Methods: Following an initial survey among uveitis experts, we collected multi-center retrospective data on 211 patients with BD uveitis and 207 patients with other uveitides, and identified ocular findings with a high diagnostic odds ratio (DOR). Subsequently, we collected multi-center prospective data on 127 patients with BD uveitis and 322 controls and developed a diagnostic algorithm using Classification and Regression Tree (CART) analysis and expert opinion.Results: We identified 10 items with DOR >5. The items that provided the highest accuracy in CART analysis included superficial retinal infiltrate, signs of occlusive retinal vasculitis, and diffuse retinal capillary leakage as well as the absence of granulomatous anterior uveitis or choroiditis in patients with vitritis.Conclusion: This study provides a diagnostic tree for BD uveitis that needs to be validated in future studies.

Investigation of clinical profile of Behçet's syndrome-related versus idiopathic branch retinal vein occlusion.

PURPOSE: To compare the long-term results of the patients with branch retinal vein occlusion (BRVO) secondary to Behçet's syndrome (BS) with the patients with unknown etiology. METHODS: Medical records and optical coherence tomography (OCT) imaging results of the patients with BRVO secondary to BS and with unknown etiology were reviewed retrospectively between 2016 and 2018 at a single center. The anatomical location of the BRVO, involvement of the macula, application of laser photocoagulation, and intravitreal injection were evaluated. RESULTS: Twenty-eight eyes of 23 patients with BRVO secondary to BS as the study group and 22 eyes of 19 idiopathic BRVO patients as the control group were included in the study. The mean duration of follow-up after the development of BRVO was 74.6 ± 57.4 months in the study group and 63.6 ± 59 months in the control group. The rate of bilaterality, macular involvement, and application of laser photocoagulation was not statistically significantly different between the groups. However, the frequency of injection requirement was significantly lower in the patients with BRVO secondary to BS in comparison to the control group (P= 0.009). CONCLUSION: Although the treatment of BRVO is laser photocoagulation and intravitreal injection of anti-VEGF agents or dexamethasone implant, the patients with BS might respond very well to systemic immunomodulatory agents in case of BRVO. Thus, rearrangement of the immunomodulatory treatment before starting intravitreal injections should be considered in the patients with BRVO secondary to BS.

Biosimilar infliximab for Behçet's syndrome: a case series.

OBJECTIVES: The efficacy and safety of biosimilar infliximab (bio-IFX) was shown in randomised controlled trials and it was approved for all indications of the reference product in several countries. However, a previous case series of 3 patients with Behçet's syndrome (BS) reported disappointing results. We aimed to share our experience with bio-IFX treatment in different types of organ involvement in patients with BS. METHODS: We reviewed the charts of all BS patients who were prescribed reference infliximab (ref-IFX) or bio-IFX in our BS clinic. Among the 181 BS patients who were prescribed IFX since 2003, 6 (3%) were prescribed bio-IFX due to refractory disease despite conventional immunosuppressives. RESULTS: A total of 6 patients (mean age: 32.1±6.2, mean disease duration: 5.3±1.8 years, 5 men and 1 woman) received bio-IFX for uveitis, nervous system, vascular and joint involvement. Four of the 6 patients obtained remission and stayed in remission during the 16±6.5 months they used bio-IFX. Among the 4 patients who obtained remission, 2 were switched to ref-IFX due to unavailability of bio-IFX infusion set and did not experience adverse events or loss of efficacy. However, relapses occurred during tapering. The other 2 patients are still in remission with bio- IFX. Among the remaining 2 patients, one had to be switched to ref-IFX after the first infusion, due to a change in the reimbursement policy and the other was non-responsive. CONCLUSIONS: Our limited experience showed that bio-IFX may be a safe and effective alternative for patients with BS, refractory to conventional immunosuppressives.

Efficacy and Safety of Sarilumab for the Treatment of Posterior Segment Noninfectious Uveitis (SARIL-NIU):: The Phase 2 SATURN Study.

PURPOSE: To assess efficacy and safety of sarilumab, a human anti-interleukin-6 receptor antibody, for treatment of posterior segment noninfectious uveitis (NIU). DESIGN: Randomized, double-masked, placebo-controlled, phase 2 study. PARTICIPANTS: Fifty-eight patients (eyes) with noninfectious intermediate, posterior, or panuveitis. METHODS: Eyes received treatment every 2 weeks for 16 weeks with subcutaneous sarilumab 200 mg or placebo. MAIN OUTCOME MEASURES: The primary end point was the proportion of patients with ≥2-step reduction in vitreous haze (VH) on the Miami scale or with a reduction of systemic corticosteroids (prednisolone or equivalent) to a dose of <10 mg/day at week 16. Primary end point was based on VH evaluation by a central reading center. Investigator evaluation of VH was a prespecified, planned secondary analysis. RESULTS: At week 16, proportion of patients taking sarilumab or placebo with ≥2-step reduction in VH or corticosteroid dose <10 mg/day was 46.1% vs. 30.0% (P = 0.2354) based on central reading center assessment of VH and 64.0% vs. 35.0% (P = 0.0372) based on investigator assessment of VH, respectively. In the subgroup of eyes with VH grade ≥2 at baseline, the mean VH reduction from baseline to week 16 was significantly greater with sarilumab vs. placebo regardless of assessment by the central reading center (-2.1 [n = 11] vs. -1.7 [n = 3], respectively; P = 0.0255) or investigator (-2.5 [n = 19] vs. -1.2 [n = 11], respectively; P = 0.0170). The mean best-corrected visual acuity gain from baseline to week 16 was greater with sarilumab vs. placebo in the overall population (8.9 vs. 3.6 letters, respectively; P = 0.0333) and in the subgroup of eyes with central subfield thickness (CST) ≥300 µm at baseline (12.2 [n = 13] vs. 2.1 [n = 7] letters, respectively; P = 0.0517). Corresponding changes in CST were -46.8 vs. +2.6 µm (P = 0.0683) in the overall population and -112.5 [n = 13] vs. -1.8 [n = 6] µm (P = 0.1317) in the subgroup of eyes with CST ≥300 µm at baseline, respectively. The most common ocular adverse events were worsening of uveitis (0 [placebo] and 3 [sarilumab] patients) and retinal infiltrates (1 [placebo] and 2 [sarilumab] patients). CONCLUSIONS: Subcutaneous sarilumab may provide clinical benefits in the management of NIU of the posterior segment, especially in eyes with uveitic macular edema.

Demographic and Clinical Characteristics of Uveitis in Turkey: The First National Registry Report.

PURPOSE: To describe the demographic and clinical profiles of uveitis patients seen at secondary and tertiary care centers in Turkey. METHODS: A nationwide web-based registry of patients with uveitis was initiated in November 2008. We analyzed data from a single baseline registry-enrollment visit. RESULTS: In 33 centers, 6967 eyes of 4863 consecutive patients were registered. The mean age at presentation was 36.6 ± 15.7 (1-92) years; 51.3% were male. Behçet disease was the leading diagnosis (24.9%), followed by ankylosing spondylitis and/or HLA-B27-associated anterior uveitis (9.7%), toxoplasmosis (7.1%), Fuchs uveitis (6.3%), and presumed herpetic anterior uveitis (6.0%). Visual acuity was 0.1 or worse in 22% of eyes. The most common ocular complications were posterior synechiae (16.2%) and cataract formation (16.2%). CONCLUSIONS: Behçet disease is still the most common non-infectious etiology in Turkish uveitis patients, while ocular toxoplasmosis and herpetic anterior uveitis are the most common infectious uveitic entities.

Infliximab for uveitis of Behçet's syndrome: a trend for earlier initiation.

OBJECTIVES: The prognosis of uveitis in Behçet's syndrome (BS) has improved over decades. Whether this is related to the use of more aggressive management strategies is not known. METHODS: This is a retrospective study of BS patients who received infliximab (IFX) for refractory eye disease between 2003-2015. The patients were divided into two groups according to the date of onset of in IFX treatment as before and after 2013. We compared the two groups in terms of disease characteristics at the onset of IFX treatment and response to treatment. RESULTS: There were 43 patients in the old and 14 patients in the new group. The duration of uveitis and previous immunosuppressive treatment before the initiation of IFX were significantly shorter in the new group compared to the old group (p=0.043 and p=0.028, respectively). The baseline visual acuity (VA) at the initiation of IFX was better in the new group, but this was only significant for the left eye. Treatment with IFX was effective in both groups in preserving VA and this was more pronounced in the new group. Attack frequency under IFX was significantly lower in the new group (p<0.001). CONCLUSIONS: IFX seems to be initiated earlier and also in less severe cases during the course of BS uveitis than before. Despite the few numbers of patients and relatively short duration of follow-up, our results give a hint that this change has improved the outcome.

Dense genotyping of immune-related loci implicates host responses to microbial exposure in Behçet's disease susceptibility.

We analyzed 1,900 Turkish Behçet's disease cases and 1,779 controls genotyped with the Immunochip. The most significantly associated SNP was rs1050502, a tag SNP for HLA-B*51. In the Turkish discovery set, we identified three new risk loci, IL1A-IL1B, IRF8, and CEBPB-PTPN1, with genome-wide significance (P < 5 × 10-8) by direct genotyping and ADO-EGR2 by imputation. We replicated the ADO-EGR2, IRF8, and CEBPB-PTPN1 loci by genotyping 969 Iranian cases and 826 controls. Imputed data in 608 Japanese cases and 737 controls further replicated ADO-EGR2 and IRF8, and meta-analysis additionally identified RIPK2 and LACC1. The disease-associated allele of rs4402765, the lead marker at IL1A-IL1B, was associated with both decreased IL-1α and increased IL-1ß production. ABO non-secretor genotypes for two ancestry-specific FUT2 SNPs showed strong disease association (P = 5.89 × 10-15). Our findings extend the list of susceptibility genes shared with Crohn's disease and leprosy and implicate mucosal factors and the innate immune response to microbial exposure in Behçet's disease susceptibility.

Temperament and Character Traits in Patients with Behçet's Disease with/without Eye Involvement.

PURPOSE: Ophthalmic involvement may lead to permanent vision loss in 25% of cases in patients with BD and it is a main concern in the literature. Although several studies have been investigated, the etiology and the cause of the disease and attacks are not yet known. This study aimed to investigate the correlation between visual impairment and personal characteristics and social circumstances in patients with BD. MATERIALS AND METHODS: A total of 153 patients with BD and age-and gender-matched 26 healthy control subjects completed the self-report Temperament and Character Inventory (TCI), Beck Depression Inventory (BDI), and State and Trait Anxiety Inventory (STAI-S and STAI-T). We classified the study participants into three groups with respect to severity of eye involvement and one control group. Each group was compared with the other two study groups and control group. RESULTS: According to TCI, we revealed that there was a trend in BD patients with eye involvement + poor prognosis having less disorderliness traits than BD patients with eye involvement + good prognosis (p = 0.016). The BD patients with eye involvement + poor prognosis had significantly lower attachment scores than BD patients with eye involvement + good prognosis (p = 0.005) and healthy controls (p = 0.005). The BD with eye involvement + poor prognosis had lower empathy scores than healthy controls (p = 0.002). In the way of average TCI parameters, only SD was statistically significant. In terms of subdimensions of TCI parameters, RD3, SD3, SD5, and C2 were shown to be statistically significant among some of the groups. CONCLUSION: BD patients with eye involvement were demonstrated to be more extravagant and socially disinterested. It may reflect that severe visual loss caused BD patients to be more systematic, depressive, self-contained, and exhausted. Considering psychological aspects of BD and its visual manifestations may contribute to helping these patients more effectively.

Safety and Efficacy of Gevokizumab in Patients with Behçet's Disease Uveitis: Results of an Exploratory Phase 2 Study.

PURPOSE: To evaluate the safety and efficacy of gevokizumab for the treatment of Behçet's disease uveitis in a prospective, open-label, randomized phase 2 trial. METHODS: Behçet's disease patients with new acute ocular exacerbation or at risk of exacerbation received 30 or 60 mg gevokizumab every 4 weeks intravenously or subcutaneously, on top of a stable regimen of immunosuppressives and corticosteroids (≤20 mg/day equivalent prednisolone). Patients withdrew in cases of ocular exacerbation. RESULTS: A total of 21 patients were included (17 acute and 4 at-risk; mean duration of uveitis 45.6 ± 37.4 months). There were no serious adverse events related to gevokizumab. Recorded adverse events were mostly associated with exacerbation of uveitis or its complications. Response was evaluated for 14 acute patients and all showed rapid control of acute ocular exacerbation, mostly within 1 week, without any increase in corticosteroid dosage. CONCLUSIONS: Gevokizumab was well tolerated and rapidly controlled acute ocular exacerbations of Behçet's disease uveitis without the need for high-dose corticosteroid.

A single endoplasmic reticulum aminopeptidase-1 protein allotype is a strong risk factor for Behçet's disease in HLA-B*51 carriers.

INTRODUCTION: Endoplasmic reticulum aminopeptidase-1 (ERAP1) protein is highly polymorphic with numerous missense amino acid variants. We sought to determine the naturally occurring ERAP1 protein allotypes and their contribution to Behçet's disease. METHODS: Genotypes of all reported missense ERAP1 gene variants with 1000 Genomes Project EUR superpopulation frequency >1% were determined in 1900 Behçet's disease cases and 1779 controls from Turkey. ERAP1 protein allotypes and their contributions to Behçet's disease risk were determined by haplotype identification and disease association analyses. RESULTS: One ERAP1 protein allotype with five non-ancestral amino acids was recessively associated with disease (p=3.13×10-6, OR 2.55, 95% CI 1.70 to 3.82). The ERAP1 association was absent in individuals who lacked HLA-B*51. Individuals who carry HLA-B*51 and who are also homozygous for the haplotype had an increased disease odds compared with those with neither risk factor (p=4.80×10-20, OR 10.96, 95% CI 5.91 to 20.32). DISCUSSION: The Behçet's disease-associated ERAP1 protein allotype was previously shown to have poor peptide trimming activity. Combined with its requirement for HLA-B*51, these data suggest that a hypoactive ERAP1 allotype contributes to Behçet's disease risk by altering the peptides available for binding to HLA-B*51.

Retinal nerve fiber layer structure abnormalities in patients with Neuro-Behcet's disease.

PURPOSE: Our aim was the evaluation of retinal nerve fiber layer (RNFL) and central macular thickness (CMT) in patients with Neuro-Behcet's disease (NBD) and to compare the results with healthy control subjects. METHODS: We recruited 50 eyes of 25 patients with NBD and 42 eyes of 21 age-matched healthy control subjects. Patients and control subjects underwent a thorough ophthalmic examination, including retinal nerve fiber layer and macular thickness measurements by optical coherence tomography. RESULTS: No significant difference was found between groups for age, sex, intra-ocular pressure and central corneal thickness measurements. The average RNFL in patients with NBD was significantly lower than that of healthy controls (86.92 ± 18.36 µm vs. 99.74 ± 8.73 µm; p = 0.00). There was significant thinning of the RNFL in three of four quadrants of the peripapillary area, superior (107.22 ± 30.91 µm vs. 125.57 ± 20.97 µm; p = 0.00), inferior (110.36 ± 25.57 µm vs. 132.19 ± 12.71 µm; p = 0.00) and nasal (68.84 ± 18.47 µm vs. 74.98 ± 11.42 µm; p = 0.05), in patients with NBD. Average CMT was significantly lower in NBD patients than in control subjects (244.06 ± 26.25 µm vs. 261.69 ± 25.71 µm; p = 0.00). CONCLUSIONS: There are significant differences in average RNFL and CMT between the two groups. RNFL and CMT thicknesses are reduced in patients with NBD compared with the healthy controls.

Ocular Involvement of Behçet's Syndrome: a Comprehensive Review.

Behçet's syndrome (BS) is a vasculitis involving several organ systems including the eyes. Ocular involvement is one of the most disabling complications of BS, causing loss of vision that may progress to blindness if left untreated. The typical form of ocular involvement is a relapsing and remitting panuveitis and retinal vasculitis. Initial attacks may spontaneously improve and subsequently disappear in a few weeks but tend to recur if left untreated. Destructive and recurrent attacks, especially with posterior segment and retina involvement, may cause irreversible ocular structural changes and permanent damage in sensory retina, resulting in loss of vision. The risk of irreversible damage to ocular tissue which may result in loss of vision warrants early and intensive treatment especially in patients at high risk such as young men who tend to follow an aggressive disease course. The management strategy involves rapid suppression of inflammation during the attacks and prevention of recurrent attacks. Local and systemic measures including immunosuppressives, corticosteroids, and biologic agents are used for this purpose. Surgery may be required in selected cases. The prognosis of eye involvement has greatly improved over the last decades with the effective use of immunosuppressives.

Colchicine in Behçet syndrome: a longterm survey of patients in a controlled trial.

OBJECTIVE: To test the hypothesis that colchicine use during early disease decreases immunosuppressive use in Behçet syndrome (BS) in the long term. METHODS: Patients with BS who participated in a double-blind, placebo-controlled trial of colchicine 16.6±1.1 years ago were evaluated for immunosuppressive use during the posttrial period. RESULTS: We could contact 90/116 patients; 28 (31%) received immunosuppressives during the posttrial period, 14 being from the colchicine arm. Posttrial colchicine use and cumulative duration were similar between patients who received immunosuppressives and those who did not. CONCLUSION: Continuous use of colchicine, even when initiated at an early disease stage, does not seem to decrease the use of immunosuppressives in the long term.

Validity and agreement of uveitis experts in interpretation of ocular photographs for diagnosis of Behçet uveitis.

UNLABELLED: Abstract Purpose: To evaluate agreement and estimate sensitivity and specificity of uveitis specialists' interpretation of ocular photographs in diagnosing Behçet uveitis. METHODS: Fourteen Turkish uveitis specialists, masked to demographic and clinical features of patients, independently labeled ocular photographs (29 Behçet/30 other diagnoses) as "Behçet uveitis" or "non-Behçet." Level of agreement was evaluated using kappa statistics. Photographs were categorized based on ocular signs captured and performance of observers. RESULTS: Exact agreement with the correct diagnosis was 56-81%. Seven reviewers correctly labeled more than 70% of photographs. Interobserver agreement among those 7 reviewers revealed moderate (κ = 0.41-0.60) or substantial (κ = 0.61-0.80) agreement in 76% of pairs. Smooth layered hypopyon, superficial retinal infiltrate with retinal hemorrhages, and branch retinal vein occlusion with vitreous haze were correctly recognized as Behçet uveitis by majority of reviewers. CONCLUSIONS: There are ocular signs of Behçet disease that can be considered diagnostic even in the absence of any other clinical information.