Vogt-Koyanagi-Harada Research Articles

Effects of pregnancy on the clinical course and treatment outcomes of Vogt-Koyanagi-Harada disease.

To describe the clinical course and treatment outcomes of Vogt-Koyanagi-Harada (VKH) disease during pregnancy. 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. 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). 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.

Advances in the Study of the Pathogenesis of Vogt-Koyanagi-Harada Syndrome.

Vogt-Koyanagi-Harada syndrome (VKHS) is a common type of uveitis characterized by the invasion of melanocyte-rich tissues. In recent years, the incidence of VKHS has been increasing yearly, and its specific pathogenesis has not yet been elucidated. However, its pathogenesis has been a hot topic of research. The clinical course of VKHS is characterized by the early involvement of the posterior segment of the eye, including exudative retinal detachment, optic papillitis, bilateral diffuse chorioretinitis, etc. If treated improperly or with delayed treatment, the inflammation may gradually spread to the anterior segment of the eye, leading to vision loss or even vision. This study examines the pathogenesis of VKHS. It reviews the progress of research on the pathogenesis of VKHS, which will help to improve the understanding of VKHS and provide a reference for subsequent studies.

Uveal Effusion Syndrome Due to WNT10A Mutation.

Uveal effusion syndrome (UES) is an exudative detachment of ciliary body, retina and choroid. Various underlying causes leads to UES-like drugs (topiramate), inflammation and hypotony. Wnt gene involvement has never been associated with UES. We report a case of bilateral UES being misdiagnosed as Vogt-Koyanagi-Harada syndrome (VKH), with Wnt gene dysfunction as the underlying trigger. A 32-year-old male presented with diminution of vision in his left eye. He was found to have choroidal detachment with retinal detachment in his left eye. Choroidal detachment was noted in the right eye. Various ocular imaging including fundus fluorescein angiography and ocular coherence tomography was done. He was misdiagnosed as a case of VKH syndrome, for which he was treated with systemic immunomodulatory therapy. However, a subclinical response was made to revisit the diagnosis. Ultrasound biomicroscopy showed supraciliary effusion. Systemic and genetic evaluation led to the detection of Wnt10A pathway mutation. UES is an entity of diagnostic challenge. Careful and thorough systemic evaluation is required to clinch the diagnosis. We reported the first case of bilateral UES recalcitrant to corticosteroids, with Wnt10A gene mutation.

Association Between Baseline Macular Morphologic Features on Optical Coherence Tomography and Visual Outcomes in Patients with Vogt-Koyanagi-Harada Disease.

The choroidal thickening and serous retinal detachments that characterize Vogt-Koyanagi-Harada (VKH) disease can be imaged in detail using spectral domain optical coherence tomography (SD-OCT). Whether specific qualitative and quantitative SD-OCT features at presentation were associated with visual outcomes in a randomized controlled trial comparing methotrexate to mycophenolate for steroid-sparing control of uveitis were evaluated. An exploratory subanalysis of data from the FAST trial in which SD-OCT images from VKH participants were analyzed for presence/absence of bacillary detachments, retinal pigment epithelium (RPE) folds, and internal limiting membrane (ILM) fluctuations was performed. A modified RPE undulation index was calculated to provide a quantifiable surrogate marker for choroidal folds. SD-OCT images were available from 158 eyes with VKH. At baseline, bacillary detachments were present in 23.5% of eyes, RPE folds in 22.8% of eyes, and ILM fluctuations in 35.2% of eyes. For each 0.1 unit increase in modified RPE undulation index, there was an associated 0.13 increase in mean logMAR BSCVA at baseline. None of the SD-OCT features were associated with BSCVA at the 6-month primary endpoint. Indeed, mean final BSCVA was similar in those with and without the SD-OCT features of interest at baseline, and was between 0.1 and 0.2 logMAR (Snellen visual acuity 20/25 to 20/30). While eyes with VKH may present with a variety of SD-OCT imaging pathology prior to starting immunosuppression with methotrexate or mycophenolate mofetil, final visual outcome in our study was excellent. With appropriate immunosuppression, good visual outcomes are possible in VKH.ClinicalTrials.gov Identifier NCT01829295Date of Registration: April 11, 2013.

Vascular Changes and Irreversible Complications in 120° Fundus Using Widefield SS-OCTA in Vogt-Koyanagi-Harada Disease.

To characterize the changes of fundus corresponding to 120° field of view in chronic Vogt-Koyanagi-Harada (VKH) disease in the quiescent phase, and explore the associations with irreversible complications in the fundus utilizing widefield swept source optical coherence tomography angiography (SS-OCTA). Prospective cross-sectional study. Sixty-nine chronic VKH patients (115 eyes) and 55 healthy controls (110 eyes) were included and underwent widefield SS-OCTA. Univariate analyses of variations in retinal and choroidal vessel density (VD), choroidal volume, and choroidal vascularity index (CVI) in VKH patients with different disease durations and the controls were conducted. Logistic regression analysis was employed to identify the associations with irreversible complications including choroidal neovascularization, vasoproliferative tumour of the retina, and chorioretinal atrophy. The Welch's analysis of variance showed lower VD of superficial retina, deep retina, choriocapillaris, and large-sized and medium-sized vessels of the choroid (LMVC), and choroidal volume and CVI in the patients with disease duration > 24 months compared to those with disease duration ≤ 24 months (all P≤0.011). The regression analysis revealed the disease duration (P=0.008; OR=1.02, 95%CI=1.005-1.035) and VD of LMVC (P=0.001; OR=0.707, 95%CI=0.575-0.87) were significantly correlated with the irreversible complications. Chronic VKH patients in the quiescent phase with disease duration > 24 months exhibit more severe decreased VD in each layer of the retina and choroid, reduced choroidal volume and sparse choroidal vascularity compared to those with disease duration ≤ 24 months. Prolonged duration and decreased VD of LMVC were associated with irreversible complications in the fundus.

Author Reply to Letter to the Editor: In Response to: Comment on Fonollosa et al.‘s “Hyper-Reflective Outer Nuclear Layer (HONL) in Vogt-Koyanagi-Harada Disease and Sympathetic Ophthalmia”

ABSTRACT We have recently described an OCT sign in two patients (one with Vogt-Koyanagi-Harada (VKH) and the other with Sympathetic Ophthalmia) consisting of hyperreflectivity of the outer nuclear layer (HONL) that subsequently evolved into outer retina atrophy and associated with poor functional outcomes. Ali et al. have published a comment on our letter regarding HONL. They have evaluated it in 90 eyes of VKH patients. It was observed in 37 eyes (41.1%) and no associations were found between HONL and structural outcomes or final visual acuity, and no cases of retinal atrophy were described. In the present author's reply, we point out two reasons for these contradictory observations. First, we considered HONL a full thickness hyperreflectivity of the outer nuclear layer, whereas they included cases with partial thickness hyperreflectivity, hence probably milder cases. Second: they have assessed visual function by means of visual acuity, so cases with extrafoveal involvement whose functional deficiency might only be measured by other tests (i.e. visual field) might have been missed.

Early and Late Treatment Influence on Chorioretinal Microvasculature in Vogt-Koyanagi-Harada Patients Using Optical Coherence Tomography Angiography.

To study the impact of early and late treatment on chorioretinal microvasculature in Vogt-Koyanagi-Harada (VKH) disease using optical coherence tomography angiography (OCTA). A total of 103 patients with VKH disease were divided into early (group 1, starting treatment within 2months after disease onset) and late (group 2, starting treatment 2months after disease onset) treatment groups. Flow area (FA) and vessel density (VD) of the retinal superficial vascular complex (SVC) and deep vascular complex (DVC), FA of the choriocapillaris, three-dimensional choroidal vascular volume (CVV), and choroidal vascularity index (CVI) were analyzed and compared to 103 healthy individuals. The relationship between the final best-corrected visual acuity (BCVA) and the aforementioned parameters was also analyzed. FA of the SVC (all P < 0.05, except 0-1mm P = 0.087), DVC (all P < 0.05), choriocapillaris (1-2.5mm P = 0.033), and CVV (all P < 0.05) were lower in group 2 as compared to group 1. Compared to healthy controls, FA of the SVC (all P < 0.001, except 0-1mm P = 0.104) and DVC (all P < 0.05), VD of the SVC (1-2.5mm P = 0.001) and DVC (1-5mm P = 0.003, 2.5-5mm P < 0.001), FA of the choriocapillaris (all P < 0.05), and CVV (total area P = 0.049, 1-5mm P = 0.045, 2.5-5mm P = 0.041) were lower in group 2, while FA (all P < 0.05, except 0-1mm P = 0.925) and VD (1-5mm P = 0.003, 2.5-5mm P = 0.004) of the DVC and FA of the choriocapillaris (total area P = 0.007, 0-1mm P < 0.001, 1-2.5mm P = 0.007) were lower in group 1. There was no significant difference concerning CVI among groups (all P > 0.05). FA of the SVC, DVC, and choriocapillaris and VD of DVC and CVI were negatively associated with the final logarithm of the minimum angle of resolution BCVA. Patients with VKH disease who are treated within 2 months of disease onset showed a better chorioretinal microvascular outcome as defined by OCTA compared to those treatedlate. Our study employs OCTA to design three-dimensional metrics for the retina and choroid, bridging the gap between traditional two-dimensional OCTA findings and enhanced clinical outcomes for patients with VKH disease.

Categories and Profiles of Uveitis in Vogt-Koyanagi-Harada Syndrome With Systemic Correlation: Inferences From a Tertiary Multispecialty Hospital.

Introduction Vogt-Koyanagi-Harada (VKH) syndromeis a granulomatous, autoimmune panuveitis, affecting the eyes, ears, skin, and meninges. It can cause choroiditisand can progress to the retina and optic disc causing visual loss. Imaging using fundus fluorescein angiography (FFA), indocyanine green angiography (ICGA), and enhanced depth imaging-ocular coherence tomography (EDI-OCT) is required for clinical evaluationand management. Steroids and immunosuppression are the treatment modalities used. Aim The aim of this study is to report the correlation and severity of uveitis in relation to systemic manifestations. Method A retrospective study including 100 patients with VKHsyndrome was carried out. They were classified based on clinical manifestations and investigations such as FFA, ICGA, B-scan ultrasonography (USG), and ocular coherence tomography (OCT). Patients were characterized as complete, incomplete, and probable VKH syndrome. Laboratory investigations were performed, and statistical analysis was done. Results Probable VKH syndrome was found to be the most common form of presentation in our study population. Defective vision was the most common complaint amongthe patients. Extraocular manifestations included tinnitus, vertigo, alopecia, headache, fatigue, and vitiligo and were seen in 33% of the patients. Disc edema and serous retinal detachment were seen in 85% of the patients. Improvement was noted in 25% of the patients with the use of corticosteroids. Conclusion Response to treatment with systemic corticosteroids and immunosuppression in the acute phase of uveitis is better compared tochronic uveitis. The ophthalmologist is usually first consulted in VKHsyndrome due to presenting ocular complaints. A multidisciplinary approach is key to providing holistic management.

Choroidal venous overload in Vogt‒Koyanagi‒Harada disease.

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. 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. 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). 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.

Dynamic Changes of Fundus and Predictors of Visual Prognosis in New-Onset Vogt-Koyanagi-Harada Disease

ABSTRACT Purpose To characterize the dynamic changes of fundus in Vogt-Koyanagi-Harada (VKH) disease through enhanced spectral-domain optical coherence tomography (EDI-OCT) and explore the predictors of visual prognosis. Methods In this retrospective cohort study, a total of 2152 VKH patients referred to our uveitis center from January 2013 to April 2022 were screened; 151 new-onset VKH patients (299 eyes) and 82 healthy controls (164 eyes) were included. The manifestations of fundus at baseline, 1 month, 3 months, and 12 months after treatment were analysed and their relevance to visual prognosis were evaluated. Results After retinal detachment (RD) (97.3%) and optic disc swelling (100%) presented at baseline, retinal reattachment (81.6%) and the granular hyperreflective depositions at the retinal pigment epithelium (RPE) (61.5%) were observed at month 1. The RPE and ellipsoid zone rearrangement accompanying interdigitation zone attenuation (57.9%) was noted finally. Choroidal thickness of patients was higher than that in the controls at baseline and month 1 (both P < 0.001). Best-corrected visual acuity (BCVA) (logarithm of the minimum angle of resolution [logMAR]) (P < 0.001; OR, 4.01), subretinal fibrinoid exudate (P < 0.001; OR, 3.9) and RPE folds (p = 0.001; OR, 2.39) at baseline, and the RD at month 1 (P < 0.001; OR, 3.42) were associated with visual prognosis. Conclusions New-onset VKH patients after treatment exhibited dynamic changes in the fundus especially the outer retina during a 12-month period. The BCVA, subretinal fibrinoid exudate, and RPE folds at baseline, and RD at month May 1, serve as predictors of visual prognosis.

Outcomes in Patients With Vogt–Koyanagi–Harada Disease From the First-Line Antimetabolites for Steroid-Sparing Treatment Uveitis Trial

PurposeTo 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. MethodsA subanalysis of patients with VKH from the First-line Antimetabolites as Steroid-sparing Treatment (FAST) Uveitis Trial, a randomized, observer-masked, comparative effectiveness trial, with comparisons by treatment (MTX versus MMF) and disease stage (acute versus chronic). Individuals with noninfectious uveitis were placed on a standardized corticosteroid taper and block randomized 1:1 to either 25mg weekly oral MTX or 1.5g 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). ResultsNinety-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). Methotrexate 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. ConclusionsMTX and MMF have comparable outcomes as corticosteroid-sparing immunosuppressive therapies for VKH. Visual acuity improvement was greater in acute vs chronic VKH. Trial RegistrationClinicalTrials.gov Identifier: NCT 0182929

Multicenter and multimodal imaging study reveals rare fundus lesions in patients after SARS-CoV-2 infection

To define the characteristics of fundus manifestations in patients after SARS-CoV-2 infection with multimodal imaging techniques. This is a retrospective multicenter and multimodal imaging study including 90 patients. All patients with a visual complaint occurring immediately after SARS-CoV-2 infection were referred to six clinics between December 2022 and February 2023. Demographic information and the temporal relationship between SARS-CoV-2 infection and visual symptoms were documented. The characteristics of the fundus lesions were evaluated using multimodal imaging. Ninety patients from six hospitals were included in this study, including 24 males (26.67%) and 66 (73.33%) females. Seventy-eight patients (86.66%) (146 eyes) were diagnosed with Acute Macular Neuroretinopathy (AMN). The AMN patients were primarily young women (67.95%). Sixty-eight patients (87.18%) had AMN in both eyes. Thirty-eight eyes (24.36%) included Purtscher or Purtscher-like lesions. optical coherence tomography and infrared retinal photographs can show AMN lesions well. Eleven cases were diagnosed with simple Purtscher or Purtscher-like retinopathy (2 cases, 2.22%), Vogt‒Koyanagi‒Harada (VKH) syndrome or VKH-like uveitis (3 cases, 3.33%), multiple evanescent white-dot syndrome (MEWDS) (2 cases, 2.22%), and rhino-orbital-cerebral mucormycosis (ROCM) (5 cases, 5.56%). After SARS-CoV-2 infection, diversified fundus lesions were evident in patients with visual complaints. In this report, AMN was the dominant manifestation, followed by Purtscher or Purtscher-like retinopathy, MEWDS, VKH-like uveitis, and ROCM.

Effect of the COVID-19 pandemic on Vogt–Koyanagi–Harada disease

To determine the disease prevalence rate and clinical characteristics of Vogt–Koyanagi–Harada (VKH) disease among new patients before and after the declaration of a state of emergency (April 7, 2020) in Japan. New patients and patients with newly diagnosed VKH disease were categorized into “Before” and “After” groups based on the initial visit. The prevalence rate, sex ratio, and age of patients newly diagnosed with VKH were compared between the groups. Best-corrected visual acuity (BCVA) and recurrence rates were compared among 59 patients observed for > 12 months after receiving pulse steroid therapy. For reference, we also examined the prevalence rate of patients newly diagnosed with acute angle closure (AAC) in the Before and After groups. The prevalence rates of VKH disease among newly diagnosed patients (P < 0.05) or patients with AAC (P < 0.001) were significantly higher in the After group. No significant differences in sex ratio or age of VKH disease were observed in both groups. BCVA and recurrence rates showed no significant differences. The COVID-19 pandemic increased the prevalence of VKH disease among new patients compared with that of AAC. However, the clinical features of VKH disease were unlikely affected by the COVID-19 pandemic.

Predictive factors and adalimumab efficacy in managing chronic recurrence Vogt-Koyanagi-Harada disease

BackgroundThis 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.MethodsA 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.ResultsA 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).ConclusionIn 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.

Development of Vogt–Koyanagi–Harada disease-like uveitis during treatment by anti-programmed death-1 antibody: a case report

BackgroundSeveral immune checkpoint inhibitors (ICIs) have been linked to the occurrence of Vogt-Koyanagi-Harada disease (VKHD)-like uveitis. Among the ICIs, there has been no report of immune-related adverse events (irAEs) caused by a new programmed death protein-1(PD-1) monoclonal antibody (Toripalimab).Case presentationThis paper presents a case of VKHD-like uveitis that arose following Toripalimab therapy for urothelial cancer of the bladder, and the patient experienced symptoms 10 days after the final dosage of 20 months of medication treatment. This patient with bladder uroepithelial carcinoma had severe binocular acute panuveitis with exudative retinal detachment after receiving Toripalimab therapy. Binocular VKHD-like uveitis was suggested as a diagnosis. Both eyes recovered after discontinuing immune checkpoint inhibitors and local and systemic corticosteroid treatment.ConclusionsThis report suggests that VKHD-like uveitis can also occur in patients receiving novel PD-1 antibodies and the importance of paying attention to eye complications in patients receiving treatment over a long period.

Pattern of uveitis in a referral ophthalmology center in Northeastern Thailand

PurposeTo report the characteristics and epidemiology of uveitis in a university-based referral center in northeastern Thailand and review the uveitis patterns present in various regions worldwide.MethodsA retrospective review of all medical records for new patients visiting the uveitis clinic at Srinagarind hospital, Khon Kaen University, between August 2016 and June 2021, was conducted.ResultsA total of 522 uveitis patients were included in this study. Disease etiologies were categorized as non-infectious 35.8% (187/522), infectious 32.8% (171/522), and undetermined cause 31.4% (164/522). Specific diagnoses were established in 68.6% of cases. Vogt-Koyanagi-Harada (VKH) (14.2%) was identified as the most common specific diagnosis, and tuberculosis (6.7%) ranked highest among infectious causes.ConclusionsAlthough non-infectious uveitis is observed predominantly in this region, the proportion of infectious uveitis is relatively more common than in developed countries. We have found no cause for one-third of our patients despite the utilization of PCR and serology for diagnostic purposes.

Multimodal Imaging in Diagnosis of Vogt Koyanagi Harada Disease with Reference to Choroid and Retinal Pigment Epithelium

A 48 year dark skinned North Indian male presented first time to our outpatient with history of bilateral visual decline of 3 weeks duration with associated features of headache, myalgia and rhinitus of 4 weeks duration. A detailed examination confirmed bilateral active anterior granulomatous uveitis with bilateral disc oedema with serous retinal detachment. Multimodal imaging examination was carried out with nideks mirante. Diagnosis in favour of vogt koyanagi harada disease was confirmed. The posterior segment was assessed for various changes in acute and remission phase of vogt koyanagi harada disease with fundus fluorescein angiography, fundus autoflourescence, retroillumination and optical coherence tomography. Thickness and structural change related to central macula thickness, retinal pigment epithelium and choroid were assessed pre and post treatment with additional support of optical coherence tomography and retroillumination. Both these techniques were able to document a greater value change pre and post treatment in these structures. Hence we conclude the need to include these techniques in retinal pigment epithelium and choroidal assessment in vogt koyanagi harada disease.

Cost-Effectiveness Analysis of Adalimumab Versus Cyclosporine for Vogt-Koyanagi-Harada Disease: A Randomized Controlled Study

Purpose To compare the 26-week cost-effectiveness of adalimumab-corticosteroids (ADA-CS) and cyclosporine-corticosteroids (CSA-CS) for Vogt-Koyanagi-Harada (VKH). Methods A preplanned cost-effectiveness analysis based on the per-protocol population of a randomized-controlled trial. VKH subjects were randomized to receive either cyclosporine (100-200 mg daily) combined with corticosteroids or adalimumab (40 mg twice monthly) combined with corticosteroids. The primary outcome of this cost-effectiveness study was the incremental cost-effectiveness ratio (ICER). Costs and quality-adjusted life-years (QALYs) data were calculated by the medical records and health utility, respectively. Subgroup (early and late-phase VKH) analysis and sensitivity analyses were performed. Results The ICER at 26 weeks was $62,425/QALY for the total participants. Compared to the CSA-CS group, costs in the ADA-CS group were more expensive (mean difference [ΔA-C]: $2,497) with more gains in QALYs (mean difference [ΔA-C]: 0.04). The probability of ADA-CS being cost-effective was 0.17 and 0.41 at willingness to pay (WTP) thresholds of $12,000/QALY and $36,000/QALY, respectively. Subgroup analysis and sensitivity analyses showed consistent findings with the primary analysis. Conclusions Regardless of early or late-phase VKH, the CSA-CS strategy may be recommended as the preferred initial choice for the majority of VKH.

Clinical and Transcriptional Profiles Reveal the Treatment Effect of Adalimumab in Patients with Initial-Onset and Recurrent Vogt–Koyanagi–Harada Disease

ABSTRACT Purpose We aimed to evaluate adalimumab efficacy in patients with initial-onset or recurrent Vogt–Koyanagi–Harada (VKH) syndrome. Methods A retrospective clinical study was performed to examine the therapeutic effect of adalimumab in 22 VKH patients,16 with initial-onset and six with recurrent VKH. Another 22 patients with initial-onset VKH who did not receive adalimumab were included as controls. The main observational parameters included the central macular thickness (CMT), subfoveal choroidal thickness (SCT), best-corrected visual acuity (BCVA), anterior chamber cell grade (ACC), glucocorticoid dose (GCD), and the development of sunset glow fundus. MRNA sequencing was used to profile the tumor necrosis factor (TNF)-α pathway in peripheral blood mononuclear cells obtained from nine patients with initial-onset VKH disease, six patients with recurrent VKH, and eight healthy controls. Results In the initial-onset group, adalimumab therapy significantly improved the BCVA, CMT, SCT, and ACC. Furthermore, adalimumab significantly decreased GCD in patients with initial-onset. In patients with recurrent VKH, the SCT significantly improved after adalimumab treatment, but no significant changes in BCVA, CMT, and ACC were observed. All six patients experienced relapse during follow-up. The TNF-α pathway exhibited a significant increase in initial-onset VKH when compared with that in both healthy controls and recurrent patients. Conversely, it was suppressed in recurrent VKH when compared with that in the initial-onset or healthy control groups. Conclusions In patients with initial-onset VKH, adalimumab effectively reduces glucocorticoid dependence. However, adalimumab may not be effective for preventing relapse or providing long-term inflammation relief in patients with recurrent VKH.

Unveiling Key Pathological Indicators for Disease Progression in Vogt Koyanagi Harada Disease and Sympathetic Ophthalmia Through Advanced Choroidal Volume Analysis

ABSTRACT Purpose To evaluate the association between quantitative parameters derived from volume analysis of optical coherence tomography (OCT) data and disease worsening in Vogt-Koyanagi-Harada disease (VKHD) and sympathetic ophthalmia (SO). Methods This retrospective study, conducted at Osaka University Hospital, employed swept-source OCT scans from patients diagnosed with VKHD or SO between October 2012 and January 2021. The choroidal vessel structure was segmented and visualized in three dimensions, generating quantitative vessel volume maps. Region-specific choroidal vessel volume (CVV), choroidal volume (CV), and vessel index (VI) were scrutinized for their potential correlation with disease severity. Results Thirty-five eyes of 18 VKHD and 2 SO patient (8 females, 10 males) were evaluated. OCT-derived CVV maps revealed regional CV alterations in VKHD and SO patients. Two parameters, i.e. CV at 3- and 6-month follow-ups (p = 0.044, p = 0.040, respectively, with area under the ROC curve of 0.70) and CVV at 6 months (p = 0.046, area under the ROC curve of 0.71), were significantly higher in recurrent VKHD and SO compared to effectively treated cases. Conclusions The volume analysis of OCT images facilitates a three-dimensional visualization of choroidal alterations, which may serve as a reflection of disease severity in VKHD and SO patients. Furthermore, noninvasive initial CVV or CV measurements may serve as potential biomarkers for predicting disease recurrence in VKHD and SO.