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Purpose: To describe the clinical course and treatment outcomes of Vogt-Koyanagi-Harada (VKH) disease during pregnancy., Methods: This retrospective study compares the clinical course and outcomes in pregnant and nonpregnant women with VKH. All VKH patients who were pregnant at presentation or on follow up and age-matched nonpregnant female controls during the study period (2013-2022) were included. Their best-corrected visual acuity (BCVA), clinical presentations, and treatment outcomes were analyzed., Results: Among the 532 female VKH patients, nine (1.7%) were included. Thirty nonpregnant patients were included as control. At presentation, the mean age in the study group was 26 years ± 6.48 standard deviation (SD) compared to the control group (mean 25.1 years ± 3.04). The mean BCVA was comparable between the two groups at presentation (study group 1.35 logarithm of the minimum angle of resolution [logMAR] vs. control group 0.76 logMAR; P = 0.05) and the final follow-up (study group 0.65 logMAR vs. control group 0.35 logMAR; P = 0.15). Participants with anterior segment inflammation (study group 77.7% vs. control group 70%; P = 0.65), the disease stage (early-stage VKH: study group 88.8% vs. control group 73.3%; P = 0.33), and disease exacerbation (study group 33.3% vs. control group 26.6%; P = 0.69) were all comparable between the study and control groups. Following treatment, 66.6% and 46.6% of the study and control groups, respectively, had sunset glow fundus ( P = 0.29). At the last follow-up, 44.4% and 26.6% of the study and control groups, respectively, had subretinal fibrosis ( P = 0.31)., Conclusion: Though pregnancy is an immunomodulatory state, the clinical course of VKH in pregnant patients can be similar to that of nonpregnant women and needs close monitoring and follow-up., (Copyright © 2024 Indian Journal of Ophthalmology.)

Purpose: To compare the effectiveness of methotrexate (MTX) and mycophenolate mofetil (MMF) in achieving corticosteroid-sparing control of uveitis in patients with Vogt-Koyanagi-Harada (VKH) disease., Methods: A subanalysis of patients with VKH from the First-line Antimetabolites as Steroid-sparing Treatment Uveitis Trial, a randomized, observer-masked, comparative effectiveness trial, with comparisons by treatment (MTX vs MMF) and disease stage (acute vs chronic). Individuals with noninfectious uveitis were placed on a standardized corticosteroid taper and block randomized 1:1 to either 25 mg weekly oral MTX or 1.5 g twice daily oral MMF. The primary outcome was treatment success defined by corticosteroid-sparing control of uveitis at 6 months. Additional outcomes included change in best spectacle-corrected visual acuity (BSCVA), retinal central subfield thickness (CST), and resolution of serous retinal detachment (SRD)., Results: Ninety-three out of 216 enrolled patients had VKH; 49 patients were randomized to MTX and 44 to MMF, of which 85 patients (46 on MTX, 39 on MMF) contributed to the primary outcome. There was no significant difference in treatment success by antimetabolite (80.4% for MTX compared to 64.1% for MMF; P = .12) or in BSCVA improvement (P = .78). MTX was superior to MMF in reducing CST (P = .003) and resolving SRD (P = .02). There was no significant difference in treatment success by disease stage (P = .25), but patients with acute VKH had greater improvement in BSCVA (P < .001) and reduction of CST (P = .02) than chronic VKH patients., Conclusions: MTX and MMF have comparable outcomes as corticosteroid-sparing immunosuppressive therapies for VKH. Visual acuity improvement was greater in acute vs chronic VKH. NOTE: Publication of this article is sponsored by the American Ophthalmological Society TRIAL REGISTRATION: ClinicalTrials.gov Identifier: NCT00182929., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Background/objectives: This study aimed to investigate the change of choroidal venous overload in Vogt‒Koyanagi‒Harada (VKH) disease. Clinical records of 52 patients with VKH disease (52 eyes) and 24 control subjects (24 eyes) who underwent multimodal imaging, including fluorescein angiography (FA) and indocyanine green angiography (ICGA), were retrospectively reviewed., Subjects/methods: Imaging data were assessed for signs associated with choroidal venous overload, e.g., choroidal perfusion delay, choroidal vascular hyperpermeability, dilated choroidal veins, and intervortex venous anastomosis (IVA). Dual FA and ICGA scoring for active posterior segment inflammation was performed. Clinical and imaging features associated with choroidal venous overload were compared between early- and late-stage VKH disease., Results: Choroidal perfusion delay, choroidal vascular hyperpermeability, dilated choroidal veins, and IVA were more prevalent in eyes with VKH disease (69.2%, 67.3%, 61.5%, and 65.4%, respectively) than in control eyes (25.0%, 20.8%, 25.0%, and 37.5%, respectively) (p < 0.05). All eyes with IVA in the early-stage of VKH disease had got other 3 signs. All choroidal venous overload signs were more prevalent in patients with early-stage (20 eyes) than in those with late-stage VKH disease (32 eyes) (p < 0.05). The number of choroidal venous overload signs were inversely related to disease duration (p < 0.001) and proportionally related to the total ICGA score (p < 0.001). IVA was significantly associated with the total ICGA score in logistic regression (p = 0.014)., Conclusions: Choroidal venous overload occurs early in VKH disease. Angiographic signs of choroidal venous overload may be useful markers to assess the status of VKH disease., (© 2024. The Author(s), under exclusive licence to The Royal College of Ophthalmologists.)

Purpose: To describe the choroidal retinal microvascular system in Vogt-Koyanagi-Harada (VKH) subjects and furnish additional proof for the early authentication and treatment of VKH suffers., Methods: From the beginning to July 2023, a comprehensive search for issued articles on optical coherence tomography angiography (OCTA) among VKH sufferers was implemented in Embase, PubMed, and Web of Science databases. This meta-analysis included 9 eligible studies. Primary endpoints included four kinds of vascular densities, such as superficial capillaris plexus (SCP), deep capillaris plexus (DCP), and choriocapillary (CC). In addition to these, there were foveal avascular zone (FAZ), central retinal thickness (CT), best-corrected distance visual acuity (BCVA log MAR), and subfoveal choroidal thickness (SFCT)., Results: SCP and DCP vessel densities in maculas were both smaller in VKH sufferers in the active stage than those normal and remission examinees (SCP vessel density, p  < 0.00001, DCP vessel density, p  < 0.00001). Compared to remission, CC vascular density was lower during the active phase. ( p  < 0.00001). SFCT and CT in the active phase exceeded those in normal and remission examinees (all of them p  < 0.00001). In terms of the patients with remission, their FAZ was bigger than that in normal subjects. (MD =0.04, p  < 0.0001)., Conclusions: Retinal and choroidal microvasculatures are characteristically changed in active VKH patients, which suggests that OCTA can be used as a tool for VKH follow-up.

Purpose: To investigate the association between pretreatment blood flow velocity in the choroid and optic nerve head (ONH) and retinal oxygen metabolism in the acute uveitic phase and the development of 'sunset glow fundus' in Vogt-Koyanagi-Harada (VKH) disease., Methods: Retrospective analysis of 41 patients (82 eyes). Laser speckle flowgraphy and retinal oximetry measurements were performed at the presentation. The main outcome measure was the development of 'sunset glow fundus'., Results: Twenty patients (40 eyes) presented in the phase preceding anterior segment inflammation (early presentation), and 21 patients (42 eyes) presented with anterior segment inflammation (late presentation). In ONH, mean blur rate (MBR)-vessel, representing blood flow velocity in retinal vessels, was significantly lower in the late presentation group, while choroidal MBR was not significantly different. The late presentation group had significantly lower oxygen saturation in retinal venules, a higher arteriovenous oxygen saturation difference and a smaller calibre of retinal arterioles compared with the early presentation group. Eyes that subsequently developed 'sunset glow fundus' had significantly lower ONH MBR-vessels, lower oxygen saturation in retinal venules, a higher arteriovenous oxygen saturation difference and a smaller calibre of retinal arterioles compared with eyes without 'sunset glow fundus'. ONH MBR-vessel had a significant negative correlation with arteriovenous oxygen saturation difference and a significant positive correlation with calibre of retinal arterioles., Conclusions: In the acute uveitic phase of VKH disease, the development of 'sunset glow fundus' is associated with pretreatment reduced retinal blood flow velocity, calibre of retinal arterioles and oxygen saturation in retinal venules, as well as an increased arteriovenous oxygen saturation difference., (© 2024 Acta Ophthalmologica Scandinavica Foundation. Published by John Wiley & Sons Ltd.)

Purpose: To evaluate changes in scleral thickness in Vogt-Koyanagi-Harada (VKH) disease., Methods: This study included 34 eyes of 17 treatment-naïve patients with acute-phase VKH disease. Scleral thickness and the presence of ciliochoroidal effusion were examined using anterior segment optical coherence tomography at baseline and 1 week, 2 weeks, and 12 weeks after the start of corticosteroid treatment. Scleral thickness was measured 6 mm posterior to the scleral spur in four directions., Results: Twenty-eight eyes (82.4%) initially had ciliochoroidal effusion, but this rapidly decreased to nine eyes (26.5%) after 1 week. The sclera with ciliochoroidal effusion became thinner from baseline to 1 week at the superior (400.2 ± 46.9-353.5 ± 47.9 µm), temporal (428.4 ± 53.6-387.8 ± 56.1 µm), inferior (451.5 ± 71.0-400.5 ± 50.5 µm), and nasal (452.4 ± 78.0-407.6 ± 62.9 µm) points (P < 0.01 for all), and no further changes were observed. The sclera without ciliochoroidal effusion remained unchanged., Conclusion: In VKH disease, eyes with ciliochoroidal effusion exhibited the maximum scleral thickness during the acute phase. This thickening responded rapidly to treatment and became thinner within 1 week. Inflammation in VKH disease may affect not only the choroid but also the sclera.

Background: This study explores prognostic factors influencing Vogt-Koyanagi-Harada (VKH) disease and observes the efficacy and safety of Adalimumab (ADA) in treating recurrence in Vogt-Koyanagi-Harada (VKH) patients., Methods: A retrospective study was conducted on all patients diagnosed with VKH disease at Beijing Tongren Hospital between 2020 and 2023. Clinical data included initial and final visual acuity, age, gender, ocular complications, treatment modalities, disease duration, and recurrence frequency., Results: A total of 62 VKH patients were included, comprising 34 in the acute-resolved group and 28 in the chronic-recurrent group. The mean age of patients in the acute-resolved group was 38.29 ± 15.46 years, while the mean age of chronic-recurrent group had a 49.00 ± 16.43 years. Initial best-corrected visual acuity (BCVA) examination at the first visit showed an average BCVA of 0.64 ± 0.29 logMAR in the acute-resolved group and 1.38 ± 0.54 logMAR in the chronic-recurrent group (p = 0.002). During follow-up, ocular complications were observed in 29.4% of the acute-resolved group patients and 41.7% of the chronic-recurrent group patients (P = 0.006). "Sunset glow fundus" was observed in 23.5% of the acute-resolved group and 64.3% of the chronic-recurrent group patients (P = 0.001). Poor initial BCVA (P = 0.046) and the occurrence of "sunset glow fundus" (P = 0.040) were significantly associated with progression to the chronic recurrent phase. Logistic regression analysis revealed that older age at onset (P = 0.042) and the occurrence of "sunset glow fundus" (P = 0.037) were significant predictors for progression to the chronic recurrent phase. ADA significantly reduced anterior chamber inflammatory cells (P = 0.000) and vitreous cavity inflammatory cells (P = 0.001) in the chronic-recurrent group, and markedly decreased the recurrence rate in VKH patients (P = 0.009)., Conclusion: In comparison to acute-resolved patients, chronic-recurrent patients exhibited poorer initial BCVA and a significantly increased incidence of "sunset glow fundus." Older age at onset and the occurrence of "sunset glow fundus" at diagnosis are crucial predictive factors for VKH patients progressing to the chronic recurrent phase. ADA effectively alleviates refractory VKH disease and is generally well-tolerated., (© 2024. The Author(s).)

Purpose: To develop a more tailored immunomodulatory treatment (IMT) strategy based on a novel 2-arm risk stratification system in Vogt-Koyanagi-Harada (VKH) patients., Design: A retrospective clinical cohort study., Methods: Seventy-nine VKH patients in the acute stage were stratified into low- (n = 58) and high-risk (n = 21) groups based on their exposure to risk factors. They were treated with oral glucocorticoids (GCs) plus as-needed (PRN) or first-line IMT. Best corrected visual acuity (BCVA), sunset glow fundus (SGF) occurrence, relapse rate, and systemic adverse events were evaluated during follow-up., Results: Compared with the low-risk group, the high-risk group showed poorer BCVA at baseline (estimated difference 0.51, 95% CI 0.30-0.78; P < .001) and 6-month follow-up (estimated difference 0.08, 95% CI 0.00-0.08; P = .006), higher incidence of SGF at 12 months (52% vs 28%; RR 1.9, 95% CI 1.1-3.4; P = .040), and higher relapse rate at 6 months (24% vs 5%; RR 4.6, 95% CI 1.2-17.5; P = .028) and 12 months (52% vs 12%; RR 4.4, 95% CI 1.9-9.7; P < .001). In the low-risk cohort, no significant difference between the 2 IMT strategies was observed in primary outcomes. In the high-risk cohort, patients with the immediate IMT showed better BCVA (estimated difference -0.20, 95% CI -0.3 to -0.08; P = .007), lower incidence of SGF (27% vs 80%; RR 0.3, 95% CI 0.1-0.9; P = .030), and lower relapse rate (27% vs 80%; RR 0.3, 95% CI 0.1-0.9; P = .030) compared with the PRN regimen. Moreover, the immediate IMT regimen had a higher frequency of systemic adverse events than the PRN regimen (47% vs 7%; RR 7.1, 95% CI 2.5-20.4; P < .001)., Conclusions: High-risk stratification at baseline was associated with poor prognosis. The immediate IMT regimen was only beneficial for high-risk VKH patients regarding visual outcome, SGF, and relapse rate. This study suggests a potential need for a customized IMT strategy for VKH patients., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Objectives: Acute central serous chorioretinopathy (CSC) and Vogt-Koyanagi-Harada (VKH) disease in the acute uveitic phase are characterized by serous retinal detachment caused by dysfunction of the choroid. The aim of this study is to compare blood flow velocity and pulse waveform parameters in the choroid between these two diseases., Methods: In this study, 25 patients (50 eyes) with VKH disease, 21 patients (27 eyes) with CSC and 15 healthy controls (30 eyes) were studied. Laser speckle flowgraphy (LSFG) was performed at presentation., Results: Choroidal mean blur rate (MBR), representing blood flow velocity in choroidal vessels, was significantly lower in the eyes affected by VKH disease compared with the healthy control and CSC eyes. CSC eyes had a significantly higher MBR compared with healthy controls. Among the analyzed pulse waveform parameters, blow-out time (BOT), falling rate (FR) and flow acceleration index (FAI) changed significantly. BOT value was significantly lower in CSC eyes than in healthy control and VKH eyes. FR and FAI values were significantly lower in VKH eyes than in healthy control and CSC eyes. There was a strong positive correlation between MBR and FAI., Conclusions: Our findings confirm different pathophysiology of these two diseases. Assessment of choroidal blood flow velocity and haemodynamics with LSFG provides useful information to differentiate acute CSC and initial-onset acute uveitis associated with VKH disease., (© 2023. The Author(s), under exclusive licence to The Royal College of Ophthalmologists.)

Aims: To investigate the changes in quality-of-life (QoL) metrics at a 24-month interval in non-acute VKHD patients and their association with inflammation, treatment, and visual function., Methods: SF-36 and VFQ-25 questionnaires were administered at two 24-month-apart moments to 22 non-acute VKHD patients followed for ≥12 months since acute disease onset. "Improvement," "unchanged," or "worsening" in questionnaires scores (difference >5-point) between M1 and M2 and their associations were sought., Results: Absence of systemic treatment or optic disc hyperfluorescence was associated with improved general health (SF-36). Improvement in binocular contrast sensitivity resulted in better ocular pain score; absence of anterior uveitis relapse, stable fundus findings, no use of cyclosporine or no intravitreal injections resulted in unchanged/better dependency score; no intravitreal injections resulted in unchanged/better mental health score (VFQ-25)., Conclusion: Stability/improvement in QoL scores was associated with controlled inflammation, better visual function, and no need for treatment. Subclinical inflammatory signs did not impact QoL scores.

Purpose: This study aimed to determine predictive factors for low final visual acuity in patients with inactive Vogt-Koyanagi-Harada (VKH) disease using Swept-source OCT and OCT-angiography (OCT-A)., Methods: We conducted a prospective longitudinal study, including 21 patients (42 eyes), who were followed up for the first 24 months after disease onset. Patients were included at the acute phase and subsequently treated. Sequential qualitative and quantitative changes in OCT and OCT-A were assessed. Analytical statistical methods were employed to determine predictive factors for final visual acuity., Results: Structural alterations including focal parafoveal outer nuclear layer atrophy, ellipsoid zone disruption, interdigitation zone disruption, and irregular and thickened retinal pigment epithelium line were observed in 57.1% of eyes at month 3, with no significant improvement over time. The presence of flow voids at months 6, 12, and 24 was significantly associated with low final visual acuity. Serous retnal detachment at presentation emerged as an independent risk factor for structural changes detected by SS-OCT during the first 2 years of the disease. Optic disc edema was predictive factor for both structural SS-OCT and OCT-A changes., Conclusion: This study underscores the importance of monitoring flow voids to predict final visual acuity and highlights the impact of serous retinal detachment at presentation on structural changes in patients with inactive VKH disease.

Objective: To investigate the correlation between serum immunoglobulin E (IgE) levels and severity of Vogt-Koyanagi-Harada (VKH) disease., Methods: The medical records of patients with VKH disease between 2015 and 2020 were reviewed. Serum immunoglobulins (IgA, IgE, IgG, and IgM), tumor necrosis factor α (TNFα) and C-reactive protein (CRP) were measured. Patients were divided into IgE-positive (IgE ≥ 100 IU/mL) and IgE-negative (IgE < 100 IU/mL) groups. The best-corrected visual acuity (BCVA) and macular morphologic characteristics including foveal thickness (FT), serous retinal detachment (SRD), sensory retinal thickness (SRT), central foveal thickness (CFT), cube volume (V), and cube average thickness (AT) were determined in patients in both groups., Results: Of 128 patients included in the study, 35 (27.34%) patients were IgE-positive, BCVA (logMAR) was worse in the IgE-positive group. The mean CRP ( P = .012) and TNFα ( P ≤ 0.001) levels were greater in the IgE-positive group than in the IgE-negative group. Regarding macular morphologic characteristics, FT ( P = .010), SDR ( P = .004), CFT ( P = .008), V ( P = .013), and AT ( P = .006) were significantly greater in the IgE-positive group than in the IgE-negative group., Conclusions: Elevated serum IgE levels were associated with more severe macular changes in patients with VKH disease. These findings suggest that IgE may be involved in the progression of VKH disease.

Purpose : To analyze quantitative OCT-angiography (OCTA) measurements of Vogt-Koyanagi-Harada (VKH) disease at different disease stages. Methods : OCTA scans of 110 VKH patients in acute uveitic, convalescent and chronic recurrent stage were reviewed; posterior uveitis recurrent cases were excluded. Superficial and deep capillary plexus (SCP and DCP) vascular densities (VD) and subfoveal choroidal thickness (SFCT) were compared. Results : DCP VD was significantly lower and SFCT higher in VKH than normal controls (NC) in all 3 stages (all p < .001). Anterior uveitis recurrent patients had lower SCP and DCP VDs and thicker SFCT than convalescent patients ( p = .001, p < .001, and p = .003, respectively). DCP VD was significantly correlated with visual acuity ( p < .05). Patients with history of anterior recurrence had lower retinal VDs in the convalescent stage than those without ( p < .001). Conclusion : Retinal microcirculation is impaired in VKH. DCP VD might be a sensitive indicator of inflammatory status and appeared to be well associated with visual outcome.

Purpose: To evaluate associations between functional and structural measurements in patients with non-acute VKHD., Methods: In this cross-sectional study, 16 non-acute VKHD patients (32 eyes; 14 female) were evaluated with multifocal electroretinogram (mfERG), standardized automated perimetry (SAP) and optical coherence tomography (OCT)examinations. All included patients had a minimum 12 months of follow-up from acute onset and were participants of an ongoing prospective study since acute phase with systematic clinical imaging evaluations and electroretinogram examinations within a predefined treatment. Age- and gender-matched controls were included. Main outcomes were functional and structural abnormalities and their correlation; secondary outcome was correlation of these findings with clinical characteristics, including fundus abnormalities., Results: SAP and mfERG parameters were significantly worse in patients than in controls. Fourteen eyes (43.7%) had disrupted ellipsoid zone (EZ); visual acuity (VA) was similar between eyes with intact or disrupted EZ. Eyes with intact and disrupted EZ differed significantly concerning N1 and P1 amplitudes and N1 peak time values on mfERG and mean sensitivity (MS), central sensitivity (CS), foveal threshold, visual field index, mean deviation (MD) and pattern standard deviation values on SAP. The area under the curve on receiver operating curves for P1 amplitude was 0.81 (cut-off value = 34.7 nV/deg 2 ) and for MD value was 0.84 (cut-off value = -5.2 dB). Central retinal thickness (CRT) significantly correlated with N1 and P1 amplitudes and P1 peak time values on mfERG (r = 0.354, r = 0.442 and r = -0.405, respectively) and MD, MS, CS and fovea threshold (log values) on SAP (r = 0.372, r = 0.406, r = 0.431 and r = 0.414, respectively). Statistically significant associations were found with the presence of peripapillary atrophy and recurrent anterior uveitis with a worse MD value (p = 0.004 and p < 0.001, respectively)., Conclusion: In non-acute VKHD, disrupted EZ and reduced CRT were correlated with impaired mfERG and SAP parameters, even in patients with good VA., (© 2020 Acta Ophthalmologica Scandinavica Foundation. Published by John Wiley & Sons Ltd.)

Purpose : To report a rare case of Vogt-Koyanagi-Harada (VKH) patient with bilateral annular choroidal detachment and describe ultra-widefield angiographic findings. Method : Case report. Results : A 61-year-old male was diagnosed as VKH based on ocular signs and neurologic findings. Annular choroidal detachment anterior to the equator was noted in both eyes. Ultra-wide field (UWF) fluorescein angiography revealed multiple leakages at posterior pole and disc with dye pooling at the margin of choroidal detachment. Late-phase UWF indocyanine green angiography (ICGA) revealed hyperpermeability with dilated veins and many vortex ampullae obscured by detached choroid. Choroidal detachment and other manifestations improved after a high-dose systemic steroid treatment. UWF ICGA after the treatment revealed decreased choroidal vessel caliber with visible vortex ampullae. Conclusion : Vortex obstruction and choroidal vein dilatation were observed in a VKH patient with choroidal detachment, which might be related to its pathophysiology.

Purpose : To demonstrate changes in three-dimensional choroidal volume with enhanced depth imaging optical coherence tomography (EDI-OCT) in patients with recurrent stage of Vogt-Koyanagi-Harada disease (VKH). Materials and Methods : This prospective comparative case series included 9 patients with recurrent VKH, 10 patients with quiet VKH, and 15 healthy controls after sample size was calculated. All VKH cases with recurrences underwent raster scanning with EDI-OCT at active and inactive stages of the disease. Results : All choroidal parameters in the active stage significantly reduced when the inflammation subsided: total choroidal volume ( P = .02), central choroidal volume ( P = .01), central choroidal thickness ( P = .03). The changes in central choroidal volume over the resolution phase were more pronounced than the changes in central choroidal thickness in 56% of cases. Two cases presenting with only subclinical posterior segment recurrence had their choroidal parameters recovered after prompt treatment. Conclusions : In the recurrent stage of VKH, alteration in choroidal volume was evident by EDI-OCT even in an absence of anterior segment inflammation. Central choroidal volume may serve as a biomarker for detecting choroidal morphological change.

Purpose : To assess the effectiveness of intravitreal (IV) bevacizumab and the need for systemic immunosuppressive therapy (IMT) in choroidal neovascularization (CNV) in Vogt-Koyanagi-Harada disease (VKHD). Methods : CNV treatment consisted of three monthly IV bevacizumab injections as a loading dose; if intra/subretinal fluid (IRF) persisted, further injections were proceeded besides increment in systemic IMT. Outcome analyses at 3, 6, and 12 months were visual acuity, central foveal thickness, macular volume, IRF, and addition of IMT. Results : Seven eyes of six patients were included. Five patients (five eyes) completed a 12-month follow-up and received 12 IV bevacizumab injections. At the 12-month follow-up, visual acuity improved in four out of five eyes ( p = .0568); all eyes had decreased macular volume ( p = .0431) but they still had persistent IRF; and all cases needed IMT introduction/increment. Conclusion : Intravitreal bevacizumab in association with systemic IMT was effective for CNV in VKHD. Active CNV may indicate disease of inadequate clinical control.

Purpose : To evaluate retinal and choroidal microvascular features of VKH patients in acute and convalescent phases after treatment using OCTA. Methods : A prospective, observational study was conducted in patients with initial VKH at the acute stage (n = 15) and healthy participants (n = 15) served as controls. After 3-month systemic corticosteroid treatment, patients' vascular parameters were recorded by OCTA before and after treatment and compared with results observed in healthy participants. Results : Our findings first uncovered that there are two types of abnormalities in the choriocapillary layer of patients with VKH in the acute stage: one is characterized as multiple dark spots of choriocapillary flow void and the other involves highly reflective areas surrounded by light spots with an increased flow area. During the convalescent stage, all eyes showed multifocal dark spots in the choriocapillary layer, leading to a reduced choroidal flow area. Conclusions : OCTA provides a better display of the microvascular appearance of the choroid to noninvasively evaluate choriocapillaris abnormalities in VKH disease.

Purpose: To clarify the chronological changes in the anterior chamber structure and identify the spherical equivalent and axial length to assess the effects of steroid pulse treatment in patients with Vogt-Koyanagi-Harada disease with active uveitis., Methods: The anterior chamber condition, including anterior chamber volume, central anterior chamber depth, peripheral anterior chamber depth, anterior chamber angle, and pupil diameter, was measured using Pentacam, and axial length was measured using IOLMaster in patients with Vogt-Koyanagi-Harada disease between June 2015 and February 2018. Furthermore, the best-corrected visual acuity, spherical equivalent, and retinal foveola thickness were also analyzed. All patients were treated with steroid pulse. All these factors were compared before and at 1 and 6 months of treatment., Results: Significant changes were observed in the anterior chamber volume, central anterior chamber depth, peripheral anterior chamber depth, anterior chamber angle, axial length, best-corrected visual acuity, spherical equivalent, and retinal foveal thickness before and at 1 and 6 months of steroid pulse treatment ( P  < 0.001, P  < 0.001, P  < 0.001, P  = 0.0015, P  = 0.027, P  < 0.001, P  = 0.0043, and P  < 0.001, respectively). No significant difference was observed in the pupil diameter before and at 1 month and 6 months of steroid pulse treatment ( P  = 0.11)., Conclusion: The anterior chamber structure, axial length, best-corrected visual acuity, spherical equivalent, and retinal foveal thickness were dramatically changed by steroid pulse treatment in patients with Vogt-Koyanagi-Harada disease who develop active uveitis. These changes were completed within 1 month.

Purpose: To assess macular capillary perfusion in patients with inactive Vogt-Koyanagi-Harada (VKH) disease by using optical coherence tomography angiography (OCTA)., Methods: A total of 51 eyes of 51 patients with inactive VKH (group 1, n = 23) and healthy volunteers (group 2, n = 28) underwent detailed eye examination including OCTA (RTVue-XR Avanti) scanning. OCTA images (6 × 6 mm) were assessed for central retinal thickness (CRT), subfoveal choroidal thickness (SFCT), vessel densities (VD) in superficial capillary plexus (SCP) and deep capillary plexus (DCP), choriocapillaris (CC) flow area and vessel flow density (VFD), foveal avascular zone (FAZ) area and acircularity index (AI)., Results: The mean ages of group 1 (13 female, 10 male) and group 2 (15 female, 13 male) were 39.9 ± 11.8 (range, 24-58) and 38.9 ± 8.5 (range, 29-51) years, respectively (p = 0.773). CRT and SFCT were 218.2 ± 39.4 μm and 195.6 ± 28.6 μm in group 1, while 243.5 ± 9.7 μm and 316.7 ± 20.1 μm in group 2, respectively (p < 0.05). VD in SCP and DCP were significantly lower in group 1 (50.6 ± 4.7% vs. 54.3 ± 3.4% and 53.9 ± 3.6% vs. 61.1 ± 2.7% respectively; p < 0.05). FAZ areas were 0.32 ± 0.11 mm 2 in group 1 and 0.25 ± 0.06 mm 2 in group 2 (p = 0.046). There was no statistically significant difference between groups regarding AI, CC flow area, and VFD (p > 0.05). There was weak negative correlation between BCVA (logMAR) and parafoveal and perifoveal VD in SCP and DCP of group 1 (p < 0.05)., Conclusion: Macular capillary perfusion was significantly reduced both in SCP and DCP in patients with inactive VKH disease.

Aims: To discover whether retinal vessel oxygen metabolism is affected in uveitis associated with Vogt-Koyanagi-Harada (VKH) disease., Methods: 41 patients with VKH disease (82 eyes) and 12 healthy subjects (24 eyes) matched in age and gender were prospectively evaluated. Retinal oxygen saturation and vessel calibre were measured with a non-invasive spectrophotometric retinal oximeter (Oxymap T1)., Results: In healthy controls, mean arteriolar oxygen saturation (%) was 93.8±5.9 and venular saturation was 60.1±5.8. In acute VKH uveitic phase associated with exudative retinal detachment (n=12), arteriolar and venular oxygen saturation values were 104.7±7.8 and 67.9±7.7, respectively, and both are significantly higher than the healthy group (p<0.001; p=0.001, respectively). In patients with VKH disease who recovered after immunosuppressive therapy and restored normal anatomy without 'sunset glow fundus' (n=13), oximetry values were 96.4±9.6 and 61.6±7.5, respectively, similar to healthy controls. In patients with 'sunset glow fundus' and chorioretinal atrophy (n=16), saturation levels were 88.6±7.8 and 50.0±13.1, respectively, significantly lower than healthy controls (p=0.02; p=0.003, respectively). These patients also had significantly smaller diameter of retinal arterioles and venules compared with controls (p=0.035; p=0.001, respectively)., Conclusions: Retinal oxygen metabolism is altered in uveitis associated with VKH disease. Oxygen saturation profile is abnormal in acute uveitic phase of the disease and returns to normal in those who recover with normal fundus appearance, but not in eyes that suffer permanent anatomical damage with 'sunset glow fundus' and chorioretinal atrophy. Retinal oximetry may be of value in evaluating vascular and metabolic aspects of posterior uveitis., Competing Interests: Competing interests: A. M. Abu El-Asrar, None; A. F. AlBloushi, None; P. W. Gikandi, None; S.H. Hardarson, Oxymap ehf. (C, I), P; E. Stefánsson, Oxymap ehf. (I, S). E. Stefánsson and S.H. Hardarson have financial interests in the retinal oximeter used in the study., (© Author(s) (or their employer(s)) 2019. No commercial re-use. See rights and permissions. Published by BMJ.)

Background: The common presence of human leukocyte antigen (HLA)-DRB1*04 in Vogt-Koyanagi-Harada (VKH) disease is well known. The aim of this study was to investigate the relationship between visual prognosis and HLA-DRB1*04 alleles during systemic corticosteroid therapy in patients with VKH disease., Methods: This retrospective case series included 57 eyes from 29 consecutive patients with treatment-naïve VKH disease who received systemic corticosteroid therapy. Visual acuity, sex, refractive error, central retinal thickness (CRT), central choroidal thickness (CCT), and duration from onset to treatment were measured at initial and final visits. Mean values of parameters were compared with each visit. Genotyping was performed by polymerase chain reaction amplification with sequence-specific primer., Results: Linear regression showed significant differences in logMAR best-corrected visual acuity between the three groups of homozygotes, heterozygotes, and normal subjects at baseline (p < 0.01), at 3 months after treatment (p < 0.01). There was no significant differences at 6 months after treatment (p = 0.257). No significant differences were detected between the three groups in age, sex, refractive error, CRT, CCT, or duration from onset to treatment., Conclusion: Alleles of HLA-DRB1*04 might affect visual prognosis and be related to early response after initiation of treatment in VKH disease.

Purpose: To prospectively evaluate the dynamic changes of the full-field electroretinogram (ff-ERG) and its association with inflammatory signs in patients with Vogt-Koyanagi-Harada disease (VKHD) followed up after acute onset., Methods: Twelve acute VKHD patients, who were followed up for at least 24 months, were enrolled at a tertiary center from June 2011 to January 2017. Treatment consisted of intravenous methylprednisolone followed by 1 mg/kg/day of oral prednisone with a slow tapering associated with late non-steroidal immunosuppressive therapy in previously defined cases. Inflammation was systematically evaluated with clinical and posterior segment imaging (PSI) exams (fluorescein angiography, FA, indocyanine green angiography, ICGA, enhanced depth imaging optical coherence tomography, EDI-OCT). A ff-ERG was performed upon enrollment as well as at predefined intervals. Scotopic ff-ERG parameters changes between the 12th and 24th months defined the ERG-stable or ERG-worsening groups. "Flare" was defined as an appearance or worsening of inflammatory signs (after the initial 6 months following disease onset) under the predefined treatment protocol., Results: ff-ERG parameters initially improved in all eyes; in the evaluation between the 12th and 24th months, ff-ERG results were stable in 17 eyes (71 %) and worsened in 7 eyes (29 %). Subnormal ff-ERG results were observed in 15 eyes (62 %) at the 24th month. On the other hand, the flare was observed in 8 eyes (33 %) as cells in the anterior chamber and in 24 eyes (100 %) as any PSI inflammatory sign. The ERG-worsening group presented thicker subfoveal choroid at the first month (p = 0.001) and fluctuations in choroidal thickness more often during follow-up when compared to the ERG-stable group (p = 0.02)., Conclusions: Scotopic ff-ERG parameters worsened between the 12th and 24th months in a quarter of the patients. Subclinical inflammation detected as an increase in CT seems to be related to worsening in visual function measured with ffERG.

Background and Objective: To evaluate the flow characteristics and textural properties of choriocapillaris (CC) on optical coherence tomography angiography in eyes with resolved inflammatory choriocapillaropathies and Vogt-Koyanagi-Harada (VKH) disease., Patients and Methods: A cohort of eyes with healed acute posterior multifocal placoid pigment epitheliopathy (APMPPE), serpiginous choroiditis (SC), and VKH disease were included. A 3 mm × 3 mm OCT angiogram of CC was acquired and graded for flow characteristics and textural properties., Results: This study included 16 patients. Texture was heterogeneous in all eyes in the SC and VKH groups, and in four eyes (40%) in the APMPPE group. Most of the eyes with VKH disease had severe low flow, whereas most of the SC and APMPPE eyes demonstrated mild low flow. Heal duration had a strong negative correlation with severity of CC low flow and a weak, statistically nonsignificant correlation with texture heterogeneity., Conclusion: Despite the resolution of active inflammation, partial CC hypoperfusion and texture disruptions persist for longer durations and may resolve in a time dependent manner. [Ophthalmic Surg Lasers Imaging Retina. 2019;50:566-572.]., (Copyright 2019, SLACK Incorporated.)

Purpose: To characterize the clinical features of sympathetic ophthalmia (SO) and compare SO and Vogt-Koyanagi-Harada (VKH) disease in Chinese patients., Design: Retrospective case series., Participants: A total of 131 consecutive SO and 500 VKH disease patients randomly selected from among those referred to our uveitis center from April 2008 through June 2018., Methods: History, extraocular and ocular findings, best-corrected visual acuity (BCVA), auxiliary examination findings, complications, and therapeutic effects were analyzed retrospectively in SO and VKH disease patients., Main Outcome Measures: Visual outcome, extraocular and ocular findings, and therapeutic effects., Results: Sympathetic ophthalmia manifested as posterior uveitis (68.8%) within 2 weeks and equal involvement of anterior and posterior segment (44.4%), respectively, was observed between 2 weeks and 2 months after disease onset. Two months after disease onset, SO patients showed sunset glow fundus (51.2%) and granulomatous anterior uveitis (27.3%). Vogt-Koyanagi-Harada disease patients mainly showed posterior uveitis (100%), anterior segment involvement (92.4%) associated with posterior uveitis (84.9%), and granulomatous anterior uveitis (97.4%) accompanying sunset glow fundus (91.5%) in the 3 periods mentioned above. The frequencies of extraocular manifestations were lower in SO patients (24.4%) as compared with VKH disease patients (84.8%; P < 0.001). Best-corrected visual acuity of SO patients improved from 0.68±0.86 logarithm of the minimum angle of resolution (logMAR) to 0.47±0.78 logMAR (P = 0.01), and BCVA of VKH disease patients improved from 0.67±0.79 logMAR to 0.24±0.53 logMAR (P < 0.001) at 12 months of follow-up. A worse BCVA was noted in SO patients compared with VKH disease patients after treatment (P = 0.003). Kaplan-Meier survival analysis showed that the risk of loss of useful vision in SO patients was significantly higher than that of VKH disease patients (P < 0.001)., Conclusions: Chinese SO and VKH disease patients have a different evolutionary process. The frequency of extraocular manifestations in SO patients is much lower as compared with VKH disease patients. Visual outcome is worse in SO as compared with VKH disease., (Copyright © 2019 American Academy of Ophthalmology. Published by Elsevier Inc. All rights reserved.)

Background: Vogt-Koyanagi-Harada (VKH) disease is a T-cell-mediated autoimmune disorder characterized by bilateral granulomatous panuveitis with various systemic manifestations. Although VKH disease rarely occurs in the pediatric population, the clinical course tends to be aggressive, and the visual prognosis is worse than that in adult patients due to severe ocular complications secondary to recurrent inflammation., Case Presentation: A 3-year-old girl with probable VKH was referred to Kobe University Hospital. She had severe bilateral panuveitis with posterior synechiae of the iris, marked optic disk swelling, and serous retinal detachment in both eyes, and her best corrected visual acuities (BCVAs) were 20/200 OD and 20/125 OS. A third course of therapy was administered because serous retinal detachment remained after two courses of therapy. She was treated with three courses of high-dose intravenous corticosteroid therapy, followed by slow tapering of oral corticosteroids. Her BCVAs recovered to 20/16 OU, and relapse of ocular inflammation and side effect of treatment were not observed during the 1.5-year follow-up period., Conclusions: We experienced a pediatric patient with probable VKH disease who was treated with three courses of high-dose intravenous corticosteroid therapy. With the favorable clinical course in our patient, initial treatment with repeated high-dose intravenous corticosteroid therapy might be beneficial in pediatric VKH disease.

Purpose: To describe the clinical features, treatment and visual outcomes of Japanese patients with new-onset acute Vogt-Koyanagi-Harada (VKH) disease., Methods: Clinical records of 111 patients who presented between 1999 and 2015 to the Ocular Inflammation Service of the Kyorin Eye Center, Tokyo, were reviewed., Results: Of the 111 patients (68 women, 43 men), 16 had complete, 90 had incomplete and 5 had probable VKH disease. The median follow-up period was 36 months (4-175 months). The mean age at presentation was 41 years (19-74 years). Serous retinal detachment (202 eyes) and optic disc hyperaemia (89 eyes) were observed at presentation. Of the patients tested, 45/48 (93.8%) were human leucocyte antigen-DR4 positive and 63/77 (81.8%) had cerebrospinal fluid pleocytosis. Initial corticosteroid treatment consisted of pulse intravenous therapy in all patients. Sunset glow fundus was observed in 49.5% of eyes, and anterior and/or posterior segment recurrence of inflammation was observed in 25 patients (22.5%). Treatment was transitioned to cyclosporine in 17 patients (15.3%) for steroid sparing (6 patients) or recurrent inflammation (11 patients), with good subsequent control. Ocular complications were observed in 47 of 222 eyes (21.2%) (mostly cataract), and systemic complications were observed in 8.1% of patients (mostly hypertension and diabetes mellitus). Ninety-three percent of eyes (167 of 178 eyes) had a visual acuity of ≥1.0 at 1 year after presentation., Conclusions: An aggressive corticosteroid treatment strategy in a large number of patients with new-onset acute VKH disease, with transitioning to cyclosporine in selected cases, resulted in excellent visual outcomes and low rates of recurrence., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2019. No commercial re-use. See rights and permissions. Published by BMJ.)

Purpose: To investigate the dynamic changes of full-field electroretinography (ffERG) and its relationship with structural changes in Vogt-Koyanagi-Harada (VKH) disease., Design: Interventional case series., Methods: A total of 42 VKH patients (84 eyes) and 45 normal controls (90 eyes) were included in this study. All of the patients showed active uveitis and were referred to us within 2 months after disease onset. Dynamic changes of retinal function were evaluated by ffERG before treatment and 2, 6, and 12 months after treatment with systemic corticosteroids and cyclosporine. The relationship between ffERG data with structural changes as disclosed by B-scan ultrasonography, fundus fluorescein angiography (FFA), indocyanine green angiography, and optical coherence tomography were also evaluated., Results: All investigated VKH patients showed a significantly decreased visual acuity and retinal structural changes before treatment. These changes significantly improved at 2 months and no abnormalities were apparent at 6 months following treatment. Full-field ERG showed significantly decreased amplitudes and prolonged implicit times for all dark-adapted (DA) and light-adapted ffERG responses before treatment. All these parameters, except the a-wave amplitude in the DA response, were improved at 2 months and returned to the normal level at 12 months., Conclusion: Our findings show that ffERG is a more sensitive method to detect improvement of visual function in VKH following treatment when compared to corrected distance visual acuity and the various imaging techniques to detect structural abnormalities in VKH., (Copyright © 2018 Elsevier Inc. All rights reserved.)

Purpose: To investigate fundus abnormalities in Vogt-Koyanagi-Harada (VKH) disease by a noninvasive tool, multispectral imaging (MSI)., Methods: A total of 77 patients with VKH and 163 healthy controls were enrolled between January and April 2015. The MSI findings were evaluated in combination with fundus fluorescein angiography (FFA), optical coherent tomography (OCT) and fundus photography (FP). Additionally, we compared extent of exposure of choroidal blood vessels between patients with VKH and healthy controls to evaluate retinal transmission function., Results: A number of features could be recognized by MSI which included (i) general depigmentation, (ii) clumping of pigment and (iii) macular depigmentation and/or hyperpigmentation. The percentages of these three abnormalities discovered by MSI in 52 inactive VKH patients with a duration of more than 2 months in VKH were 67.3%, 75% and 84.6% respectively, which were all significantly higher than those detected by FFA, OCT or FP (p = 0.0398, p < 0.0001). Our study showed that MSI detected a higher percentages of these four abnormalities in patients with sunset glow fundus than in patients without this phenomenon (p = 0.0492, p < 0.0001). Additionally, at the same wavelength (590 nm), exposure of choroidal blood vessels in inactive patients was stronger than in healthy controls and active patients (p < 0.01, p < 0.001)., Conclusion: Our results show that MSI is a sensitive noninvasive method to investigate the retinal pigment epithelium (RPE) in VKH disease. It is more likely to detect RPE abnormalities with MSI than when using FFA, OCT or FP. Retinal transmission function in inactive patients is stronger than that observed in active patients or healthy controls., (© 2017 Acta Ophthalmologica Scandinavica Foundation. Published by John Wiley & Sons Ltd.)

BACKGROUND This study analyzed the macular 3D-OCT images of Vogt-Koyanagi-Harada disease (VKH) in uveitis, explored the characteristics of 3D-OCT images of the macular region of VKH, and assessed which characteristics contribute most to VKH diagnosis. MATERIAL AND METHODS The 3D-OCT examination of 25 cases of VKH was performed on the macular area, and the image characteristics were analyzed. RESULTS Our study included a total of 50 eyes from 25 cases of VKH patients, 10 males and 15 females, aged 17 to 64 years, mean (39.44±11.60) years old. According to OCT B-scan images, 49 (98%) eyes had ERD, 49 (98%) eyes had nerve retinal edema, 36 (72%) eyes had endometrium-like structure (including cysts), 5 (10%) eyes had RPE folds, 35 (70%) eyes had changes in the internal septum, 49 (98%) eyes had RPE monolayer structure outside the ERD region. In ILM-RPE thickness, 49 (98%) eyes had retinal irregular thickening and 31 (62%) eyes had radial stripe changes. In ILM contour figure, 50 eyes (100%) showed exceptional uplift, 5 (10%) eyes had small focal uplift for PED on the RPE surface, and 48 (96%) eyes had wavy ups and downs. CONCLUSIONS In OCT B-scan imaging, the ERT, retinal edema of the retina, and the RPE monolayer structure outside the range are most likely to occur in VKH. The ILM-RPE thickness chart in 3D reconstruction showed irregular thickening of the retina. The ILM contour graph showed abnormal uplift, and RPE surface wavy ups and downs in VKH most likely to occur.

Purpose: Our purpose was to study changes in macular cone photoreceptors in Vogt-Koyanagi-Harada (VKH) disease patients after high-dose corticosteroid treatment using an adaptive optics (AO) fundus camera., Methods: We retrospectively analyzed 16 eyes of eight patients with new-onset acute VKH disease that were studied retrospectively. After serous retinal detachment (SRD) had resolved, AO images were obtained using the rtx1™ AO fundus camera over a 12-month course. Cone counting was performed in the nasal, temporal, superior and inferior areas of the macula 0.75 mm from the foveal center. A control group of 30 eyes of 30 healthy subjects was established for comparison., Results: In the eyes with VKH disease, the mean cone densities 0.75 mm from the foveal center were 11,847 at baseline (resolution of SRD), and 15,218, 16,684 and 17,686 cones/mm 2 , at 3, 6, and 12 months later, respectively. The mean cone densities at 3, 6, and 12 months were significantly increased compared to those of baseline (p = 0.007, p < 0.001, and p < 0.001, respectively); however, they were significantly lower than that of the healthy control eyes (p < 0.001). The mean cone densities at areas with a previous presence of cystoid spaces were significantly lower than those without cystoid spaces at the baseline, and at 3, 6, and 12 months (p < 0.001, p = 0.007, p < 0.001, and p = 0.008, respectively)., Conclusions: Cone densities were gradually increased after the resolution of SRD in the eyes of VKH disease patients. The presence of cystoid spaces might be a marker of severe damage to cone photoreceptors.

Purpose: To investigate the effectiveness, visual outcome, and prognostic factors of Vogt-Koyanagi-Harada (VKH) disease treatment with a reduced dose of corticosteroids combined with immunosuppressive agents., Methods: The clinical characteristics, auxiliary examinations, treatment result, visual outcome, and prognostic factors in VKH patients were analyzed., Results: A total of 998 VKH patients were divided into posterior uveitis group (Group1), anterior uveal involvement group (Group 2), and recurrent granulomatous anterior uveitis group (Group 3). Reduced doses of corticosteroids combined with immunosuppressive agents were used for 1-1.5 years. Uveitis was controlled in 100%, 100%, and 96.8% of these three groups, respectively. Visual improvement and stability was observed in 98.1%, 96.5%, and 88.3%, respectively. Treatment after disease onset, visual acuity at first visit, and 1 month after treatment was positively associated with BCVA at last visit (p < 0.05)., Conclusion: A reduced dose of corticosteroids combined with immunosuppressive agents effectively controlled the intraocular inflammation and improved visual acuity in most Chinese VKH patients.

Purpose: To evaluate the clinical outcome and safety of adalimumab in patients with Vogt-Koyanagi-Harada (VKH) disease., Methods: VKH patients treated with adalimumab seen at the University of Buenos Aires were reviewed. Main outcome measures were visual acuity, anterior segment inflammation, optic nerve inflammation (ONI), steroid sparing effect, number of immunosuppressives, and relapses., Results: In total, 14 VKH patients, mean age 23.07 ± 8 years; median of adalimumab treatment 10 months, were analyzed. At start of adalimumab treatment (baseline), median of corticosteroid dose was 20 mg and at 6 months, 4 mg. At baseline, 11 patients were on immunosuppressive treatment and at 6 months only four continued with immunosuppressive therapy. In the 28 eyes, the median of active inflammation was 2 at baseline and 0 after 6 months on adalimumab., Conclusions: Treatment with adalimumab is an effective and safe option, reducing the need for oral corticosteroid and conventional immunosuppressive therapy.

Purpose: To measure changes in the choroidal vascularity index (CVI) in chronic Vogt-Koyanagi-Harada (VKH) disease during a recurrent anterior uveitis attack., Methods: Forty VKH patients and 40 normal controls were included in this study. Choroidal images were recorded before and during a recurrent anterior uveitis attack, as well as after appropriate treatment. CVI was measured by the binarization technique using ImageJ software (Bethesda, MD)., Results: The CVI was 0.75 ± 0.09 in quiescent VKH patients, which was significantly higher compared to healthy controls (0.70 ± 0.05, p ＜ 0.0001). The CVI significantly decreased to 0.72 ± 0.09 when granulomatous anterior uveitis appeared in these patients. However, it returned to 0.75 ± 0.08 after uveitis resolved., Conclusions: A significant decrease of the CVI occurred during recurrent anterior uveitis in chronic VKH. CVI may provide a novel parameter to guide the treatment of VKH disease.

Purpose: To describe surgical outcomes of macular detachment caused by subretinal fibrosis (SRF) in Vogt-Koyanagi-Harada disease (VKH)., Methods: Retrospective review of VKH patients who underwent SRF removal surgery., Results: Seven eyes of six VKH patients with preoperative BCVA ranging from light perception to 20/250 were included. Six eyes underwent uncomplicated SRF removal with C3F8 or silicone oil (SO) tamponade and the following optional primary or subsequent procedures: intravitreal injection of triamcinolone acetonide, SO removal, lensectomy, or phacoemulsification with intraocular lens (IOL) implantation. All six eyes had attached macula and improved BCVA at the last visit (ranging from 20/2000 to 20/67) compared to baseline; the other eye, however, showed no light perception after surgery due to optic nerve injury., Conclusions: In VKH patients, macular detachment caused by SRF can be treated with surgery with generally favorable outcomes. Extreme caution should be taken to avoid optic nerve injury.

Purpose: To describe the clinical presentation of Vogt-Koyanagi-Harada (VKH) syndrome in a group of patients in Colombia., Methods: Retrospective review of 2638 medical records of patients with uveitis in two centers during 17 years., Results: A total of 25 patients with uveitis were diagnosed with VKH syndrome (0.95%), 23 patients were included in the data analysis (0.87%), 78.3% females, and mean age of diagnosis was 37 years (SD ± 29). Main complaints: blurred vision (87%), headaches (47.8%), tinnitus (26.1%), and hearing impairments (21.7%). Ophthalmic findings: bilateral serous retinal detachment (73.9%) and non-granulomatous uveitis (52.3%). Most of the patients were diagnosed with probable disease (56.5%). Mean duration of follow-up was 14 months; disease relapse was encountered in 26% of patients despite treatment., Conclusion: Patients in Colombia with VKH had clinical features similar to those reported in other Hispanic populations, except for the non-granulomatous uveitis. This disease may be considered as having variation of clinical manifestations across population groups.

We here report the case of a 27-year old patient, followed-up in our Department for treatment of chronic Vogt-Koyanagi-Harada disease ( VKH disease). Fundus examination showed depigmentation of the retinal pigment epithelium and of the choroid, appearing as a pseudotumoral peripapillary lesion. Vogt-Koyanagi-Harada disease is a multisystem disorder, characterized by bilateral granulomatous panuveitis with serous exudative multifocal retinal detachment. Pathophysiology of this disease is unknown, but an immunological cellular reaction against melanocytes of the skin, the meninges, the retina, the uvea, the cochlea and the labyrinth is suspected. This disease mainly occurs in young subjects from the Far East as well as in pigmented subjects. Ocular involvement is often associated with neurological (meningeal stiffness, headache, sometimes associated with focal deficit and erebrospinal fluid (CSF) pleocytosis), auditory ( perceptive deafness) and cutaneous (vitiligo, poliosis, alopecia and canities) manifestations. It usually evolves in three phases: a prodromal phase mainly characterized by neurological signs, an acute uveitic phase, a chronic phase of convalescence characterized by choroidal and tegument depigmentation or a phase of recurrence during which subretinal neovessels and subretinal fibrosis may appear. Scarrings manifest during the chronic phase of VKH disease, which is dominated by diffuse depigmentation of the fundus of the eye, scars due to nummular chorioretinal atrophy, wheals due to diffuse depigmentation, macular scar remodeling. Pseudotumoral appearance is rare and atypical during the chronic phase of VKH disease. Treatment is based on intravenous corticosteroids followed by a cycle of oral therapy. Patient should be early treated with massive and prolonged therapy to improve prognosis.

Purpose: To investigate prognostic factors for retinal sensitivity assessed by microperimetry in patients with Vogt-Koyanagi-Harada (VKH) disease., Methods: In total, 34 patients with initial-onset acute disease and 19 patients with chronic recurrent disease were retrospectively evaluated., Results: The mean follow-up period was 40.4 ± 40.5 months. Sensitivity was significantly worse in eyes with more severe anterior segment inflammation at presentation, as indicated by the presence of mutton-fat keratic precipitates, anterior chamber reaction ≥2+, and posterior synechiae. Chronic recurrent presentation, development of complications, and 'sunset glow fundus' were significantly associated with worse sensitivity. Using logistic regression analysis, better sensitivity was significantly associated with initial-onset acute presentation (odds ratio, OR = 6.9; 95% confidence interval, CI = 1.53-9.66)., Conclusions: Chronic recurrent presentation and development of complications and 'sunset glow fundus' are associated with a worse sensitivity outcome.

Purpose: To determine if the inflammatory composition of subretinal fluid in Vogt-Koyanagi-Harada (VKH) serous retinal detachments is predictive of photoreceptor injury, and to quantify photoreceptor recovery, following resolution of these detachments., Methods: Optical density (OD) measurements of spectral-domain optical coherence tomography (SD-OCT) scans were used to derive the fibrinous index, a measure of the inflammatory composition of subretinal fluid. In order to assess photoreceptor status, photoreceptor outer segment (PROS) volume was measured from SD-OCT scans., Results: The fibrinous index of subretinal fluid in VKH uveitis was strongly correlated with the PROS volume following resolution of subretinal fluid (r = -0.70, p = 0.006). Following fluid resolution, both PROS volume (p < 0.0001) and visual acuity (p = 0.0015) improved., Conclusions: The fibrinous index of subretinal fluid during the acute stage of VKH can predict photoreceptor status following resolution of subretinal fluid. PROS volume is a useful measure of photoreceptor recovery in VKH.

Purpose: To determine the effect of low-dose cyclosporine (CyA) treatment for patients with chronic Vogt-Koyanagi-Harada (VKH) disease resistance to systemic corticosteroid treatment. Methods: We retrospectively evaluated patients diagnosed with chronic VKH disease resistance to systemic corticosteroid treatment at Japan Community Health Care Organization (JCHO) Osaka Hospital between March 2013 and March 2016. We followed the observation with systemic low-dose CyA (100 mg once daily) treatment of these patients. The patients were divided into two groups, anterior ocular inflammation group and posterior ocular inflammation group. Demographic data were reviewed, including age, gender, the existence of inflammation at the initial visit and three months after CyA treatment, and side effect. Results: Twenty-three eyes of thirteen patients with chronic VKH disease were included in this study (11 women, 2 men; mean age, 54.6±11.9 years). Nine cases showed anterior ocular inflammation and seven cases showed posterior ocular inflammation (includes overlapping cases). Thirteen of fourteen eyes in the anterior ocular inflammation group subsided at three months after CyA treatment, and ten of thirteen eyes in the posterior ocular inflammation group subsided at three months after treatment. We had to stop the treatment in one patient because of severe increase of serum triglyceride. Conclusions: Low-dose CyA treatment was effective in patients with chronic VKH resistance to systemic corticosteroid treatment. Our results suggest that this treatment was more effective in the anterior ocular inflammation group than in the posterior ocular inflammation group; however, a larger number of patients and longer observation periods are needed to confirm these results.

Purpose: To evaluate the effectiveness and safety of mycophenolate mofetil (MMF) as first-line therapy combined with systemic corticosteroids in initial-onset acute uveitis associated with Vogt-Koyanagi-Harada (VKH) disease., Methods: This prospective study included 38 patients (76 eyes). The main outcome measures were final visual acuity, corticosteroid-sparing effect, progression to chronic recurrent granulomatous uveitis and development of complications, particularly 'sunset glow fundus'., Results: The mean follow-up period was 37.0 ± 29.3 (range 9-120 months). Visual acuity of 20/20 was achieved by 93.4% of the eyes. Corticosteroid-sparing effect was achieved in all patients. The mean interval between starting treatment and tapering to 10 mg or less daily was 3.8 ± 1.3 months (range 3-7 months). Twenty-two patients (57.9%) discontinued treatment without relapse of inflammation. The mean time observed off of treatment was 28.1 ± 19.6 months (range 1-60 months). None of the eyes progressed to chronic recurrent granulomatous uveitis. The ocular complications encountered were glaucoma in two eyes (2.6%) and cataract in five eyes (6.6%). None of the eyes developed 'sunset glow fundus', and none of the patients developed any systemic adverse events associated with the treatment., Conclusions: Use of MMF as first-line therapy combined with systemic corticosteroids in patients with initial-onset acute VKH disease prevents progression to chronic recurrent granulomatous inflammation and development of 'sunset glow fundus'., (© 2016 Acta Ophthalmologica Scandinavica Foundation. Published by John Wiley & Sons Ltd.)

Vogt-Koyanagi-Harada (VKH) disease is an idiopathic, multisystem autoimmune disorder characterized by bilateral, diffuse granulomatous uveitis associated with neurological, audiovestibular, and dermatological manifestations. The purpose of this study is to investigate vestibular functions in patients with VKH disease. A total of 43 patients with VKH disease in Hokkaido University Hospital were enrolled in this study. Subjective symptoms such as dizziness or vertigo and the results of various vestibular examinations including nystagmus testing, caloric testing, and vestibular-evoked myogenic potential (VEMP) testing were investigated. Eight of 42 patients (19.0%) complained of subjective vestibular symptoms. On the other hand, 12 of 28 patients (42.9%) showed nystagmus, and 7 of 15 patients (46.7%) showed unilateral or bilateral weakness in the caloric test. VEMP testing was performed for 16 patients. Seven (43.8%) and 8 (50.0%) patients were evaluated as abnormal in cervical VEMP and ocular VEMP testing, respectively. The rate of detection of nystagmus was significantly higher than that of subjective symptoms. As vestibular dysfunction in patients with VKH disease cannot be detected through history taking alone, nystagmus testing, caloric testing, and VEMP testing should be performed to evaluate vestibular functions associated with VKH disease. It is considered that abnormal VEMP findings are associated with otolith organ dysfunction., (© 2017 S. Karger AG, Basel.)

Purpose: To assess choroidal inflammation-related circulatory changes associated with the anterior recurrence of Vogt-Koyanagi-Harada (VKH) disease, using indocyanine green angiography (ICGA) and laser speckle flowgraphy (LSFG)., Methods: This retrospective case series included 17 eyes of 11 patients with VKH disease showing recurrent inflammatory findings in the anterior, but not posterior, segment (i.e. anterior recurrence). Indocyanine green angiography (ICGA) and LSFG were performed at the time of recurrence and one month after the initiation of corticosteroid therapy. The number and total area of hypofluorescent dark dots (HDDs) on ICGA were independently counted by three physicians and measured with ImageJ, respectively. Mean blur rate (MBR), a quantitative index of relative blood flow velocity, was calculated via the LSFG Analyzer software., Results: Hypofluorescent dark dots (HDDs) were identified on ICGA in 13 of 17 eyes (76%) with the anterior recurrence of VKH disease. The number and total area of HDDs significantly decreased from 203 ± 101 dots to 59 ± 51 dots and from 48 789 ± 24 251 pixels to 15 664 ± 13 254 pixels, respectively. The change ratio of MBR significantly increased by 17.9 ± 16.3% after the treatment. Importantly, there was no significant association between the change ratios of HDDs and MBR., Conclusions: These findings on LSFG and ICGA clearly demonstrated subclinical involvement as well as post-treatment improvement of choroidal circulation impairment due to granulomatous inflammation in eyes with the anterior recurrence of VKH disease. The present data suggest the validity of using these two examinations, capable of detecting different circulatory changes, in the management of recurrent VKH disease., (© 2016 Acta Ophthalmologica Scandinavica Foundation. Published by John Wiley & Sons Ltd.)

Purpose: To describe the long-term outcomes of patients with limited Vogt-Koyanagi Harada syndrome (VKH), characterized by steroid-responsive serous retinal detachments without other signs of intraocular inflammation., Design: Retrospective case series., Methods: Consecutive patients from the Royal Victorian Eye and Ear Hospital with acute bilateral serous retinal detachments without anterior chamber inflammation, with no previous ocular history or trauma, and with negative screening for vasculitis and other systemic autoimmune disease were included. Serous retinal detachment was confirmed on optical coherence tomography (OCT) and fundus fluorescein angiography. Visual acuity, treatment regimen, complications, and duration of follow-up were recorded., Results: Nine patients (6 male, median age 29 years [interquartile range (IQR) 27-36 years]) from January 2010 through May 2014 were studied. Median presenting logMAR visual acuity (VA) was 0.48 (IQR -0.1 to 3.0). Six patients were initially commenced on intravenous methylprednisolone for 3 days, followed by oral prednisolone. All received a course of tapering oral prednisolone (1 mg/kg prior to taper). Median duration of treatment was 9 weeks (IQR 8-14 weeks). Median time to complete resolution of subretinal fluid on OCT was 3.7 weeks (range, 2-12 weeks), with a corresponding improvement in VA (median logMAR 0.00; range, -0.08 to 0.04). There was no recurrence after a mean follow-up of 145.2 weeks (95% confidence interval 72.5-217.9)., Conclusion: In contrast to typical VKH, patients with limited VKH in our series appear to have good outcomes with systemic corticosteroid treatment. They have a marked improvement of VA and no episodes of relapse after cessation of corticosteroid treatment., (Copyright © 2016 Elsevier Inc. All rights reserved.)

Objectives: To evaluate the outcomes of trabeculectomy with mitomycin-C (MMC) in uveitis associated with Vogt-Koyanagi-Harada disease (VKH)., Methods: Retrospective cohort study included 27 eyes of 15 patients with uveitic glaucoma associated with VKH disease who underwent trabeculectomy with MMC as a first glaucoma procedure at King Khaled Eye Specialist Hospital, Riyadh, Saudi Arabia between 1992 and 2011. The main outcome measures were: the intraocular pressure (IOP), the number of antiglaucoma medications, the presence of visually threatening complications, and the need for further surgeries to control the IOP., Results: The mean follow-up was 78.19 months. The IOP decreased significantly from a mean of 40.15±7.0 to 13.12±6.9 mm Hg on the last follow-up (P<0.01) as well as during the whole follow-up period. The number of medications needed to control the IOP also decreased significantly from 3.89±0.6 to 1.04±1.3. The cumulative probabilities of success were 77.8%, 74.1%, 63.0%, and 51.9% at 12, 24, 36, and 48 months postoperatively, respectively. The most common complications were cataract development and progression (53.8%), hypotony (IOP≤5 mm Hg) (33.3%), choroidal effusion (7.4%), and decompressive retinopathy (7.4%). Eight eyes (29.6%) needed a second procedure to control the IOP., Conclusions: Trabeculectomy with MMC offers an acceptable intermediate term success and IOP control in uveitic glaucoma associated with VKH. However, significant number of eyes needed further procedures to control the pressure. Therefore, continuous monitoring of the pressure is important.