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

Purpose: To study the impact of early and late treatment on chorioretinal microvasculature in Vogt-Koyanagi-Harada (VKH) disease using optical coherence tomography angiography (OCTA). Methods: A total of 103 patients with VKH disease were divided into early (group 1, starting treatment within 2 months after disease onset) and late (group 2, starting treatment 2 months 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. Results: FA of the SVC (all P < 0.05, except 0-1 mm P = 0.087), DVC (all P < 0.05), choriocapillaris (1-2.5 mm 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 0-1 mm P = 0.104) and DVC (all P < 0.05), VD of the SVC (1-2.5 mm P = 0.001) and DVC (1-5 mm P = 0.003, 2.5-5 mm P < 0.001), FA of the choriocapillaris (all P < 0.05), and CVV (total area P = 0.049, 1-5 mm P = 0.045, 2.5-5 mm P = 0.041) were lower in group 2, while FA (all P < 0.05, except 0-1 mm P = 0.925) and VD (1-5 mm P = 0.003, 2.5-5 mm P = 0.004) of the DVC and FA of the choriocapillaris (total area P = 0.007, 0-1 mm P < 0.001, 1-2.5 mm 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. Conclusions: 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 treated late. Translational Relevance: 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.

CHANGES IN SCLERAL THICKNESS IN THE ACUTE PHASE OF VOGT-KOYANAGI-HARADA DISEASE.

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.

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.

Bilateral peripapillary choroidal neovascular membranes in Vogt-Koyanagi-Harada disease.

Choroidal neovascular membrane (CNVM) in Vogt-Koyanagi-Harada disease (VKH) is a known entity, observed primarily during the chronic convalescent and chronic-recurrent phases of the disease. However, the peripapillary location of CNVM is a rare finding.We describe a case of chronic VKH with bilateral peripapillary CNVM detected using multimodal imaging and the associated differential diagnoses and treatment approach.A combination of anti-vascular endothelial growth factor injections, systemic steroids and immunosuppressants is often required to manage the aggressive course of this choroidal neovascularisation.

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

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.

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

BACKGROUND: Several 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 PRESENTATION: This 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. CONCLUSIONS: This 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.

Surgical management of a case of non-resolving bullous retinal detachment due to probable Vogt-Koyanagi-Harada syndrome.

A 45-year-old male with Vogt-Koyanagi-Harada (VKH) syndrome presented with vision loss in his right eye after discontinuing treatment during the COVID-19 pandemic. He was found to have bullous retinal detachment (RD) in the right eye and was started on subcutaneous adalimumab with oral corticosteroid following three doses of pulse corticosteroid. But when RD did not resolve after 4 months of treatment and ultrasound B scan showed bullous RD with retino-retinal adhesion, he was planned for surgical intervention. During surgery, there was retino-retinal adhesions due to long-standing "kissing exudative RD", causing non-settling exudative RD. Following surgery, the vision improved to 2/60, with attached retina. The patient has been under follow-up with us for the last one year now and developed no recurrence of RD till now. This case emphasises the significance of retinoretinal adhesion in long-standing bullous RD that does not respond to conventional aggressive medical therapy.

Vogt-Koyanagi-Harada disease after SARS-CoV-2 infection: Case report and literature review.

BACKGROUND: Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) is responsible for coronavirus disease 2019 (COVID-19), a complex and multifaceted illness. COVID-19 is associated with various ocular manifestations including conjunctivitis, retinal vein occlusion and optic neuritis. However, the case of Vogt-Koyanagi-Harada (VKH) disease associated with SARS-CoV-2 is infrequent, and the specific association is still unclear. CASE PRESENTATION: In the present study, a 35-year-old female patient without any significant medical history presented with 1 week of bilateral blurred vision, occurring 2 weeks after a clinical course of COVID-19. Upon examination, both eyes exhibited bullous serous retinal detachments. She was diagnosed with incomplete VKH disease. Early diagnosis and treatment of VKH disease are essential for the visual prognosis of this aggressive disease. In this particular patient, ocular inflammatory signs and visual acuity improved via corticosteroid therapy. It is worth noting that the occurrence of VKH disease associated with SARS-CoV-2 is uncommon, and the specific connection between the two remains unknown. We review and summarize the clinical characteristics of VKH disease following SARS-CoV-2 infection, and discuss the potential mechanisms that may explain this phenomenon, based on similar studies previously reported. CONCLUSION: Despite the unclear causality, it is important for ophthalmologists and physicians to be recognizant of the possible association between VKH disease and COVID-19. SARS-CoV-2 may play a potential immunological triggering role in VKH disease. However, further in-depth research is necessary to investigate the clinical and epidemiological features, as well as the underlying mechanisms of this association.

A case report: co-occurrence of probable Vogt-Koyanagi-Harada disease and diabetic retinopathy.

BACKGROUND: Bilateral retinal detachment and choroidal detachment in a patient are rare occurrences. The presence of bilateral diabetic retinopathy (DR) in such a case is even rarer and complicates the condition. CASE PRESENTATION: In this study, we document a case of unconventional VKH. Manifestations in this patient included intense peripheral retinal detachment and choroidal detachment, along with vitreous opacities akin to cotton wool spots, concurrent with DR. The diagnosis was considered as probable VKH with DR. Treatment according to VKH protocols, including high-dose corticosteroids, yielded positive results. CONCLUSIONS: VKH can co-occurrence with DR. VKH manifestations vary, and early, aggressive, and long-term treatment is essential. The complexity of treatment increases with concurrent DR, necessitating the use of immunosuppressants.

[Literature data mining on the current research status of uveitis in China].

Objective: To analyze the current research status of uveitis in China. Methods: It was a bibliometric analysis study. Using search formulas covering uveitis and its multiple subtypes, uveitis-related literature in English with publication dates from 2013 to 2022 was retrieved in Web of Science core databases through certain search strategies. This study used the latent Dirichlet allocation (LDA) algorithm to build topic models and analyzed the trends of research topics in recent years. Bibliometric analysis was used to analyze and visualize the bibliometric indicators (e.g., number of publications, citations, and H-index) of the included literature using tools such as VOSviewer software. Results: Over the past decade, China has published 1 657 papers on uveitis, ranking second globally. However, there is still room for improvement in terms of the H-index (58) and citation (12.28 per publication). Countries such as the USA (43.04%) and the United Kingdom (62.54%) were engaged in more international collaboration. We identified ten optimal LDA topics for uveitis literature in China such as immunotherapy, Behçet's disease, and Vogt-Koyanagi-Harada syndrome. Research on uveitis in China was mostly published in Ocular Immunology and Inflammation (92). Conclusions: China has made remarkable progress in uveitis research. Nonetheless, there is still untapped potential to enhance our global academic influence. It is encouraged to promote international collaborations, harness our expertise in areas like Behçet's disease and VKH syndrome, and publish our scientific achievements in high-impact journals.

Optical Coherence Tomography Angiography in Vogt-Koyanagi-Harada Patients: A Systematic Review and Meta-Analysis.

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.

Pretreatment ocular blood flow and retinal oxygen metabolism in the acute uveitic phase is associated with final outcome in Vogt-Koyanagi-Harada disease.

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.

Vogt-Koyanagi-Harada disease developed during chemotherapy for Hodgkin lymphoma: a case report.

BACKGROUND: Ocular manifestations are known for non-Hodgkin lymphoma, but are rare for Hodgkin lymphoma. We report a case of Vogt-Koyanagi-Harada (VKH) disease presenting as serous retinal detachment and uveitis in both eyes in a child undergoing chemotherapy for Hodgkin lymphoma. CASE PRESENTATION: The patient was a 7-year-old boy with stage IIB Hodgkin lymphoma (nodular lymphocyte predominant type) who was undergoing chemotherapy, including 2 cycles of the OEPA regimen and 1 cycle of the COPDAC regimen. Two days after the end of the COPDAC regimen, the patient complained of headache and of blurred and decreased vision in both eyes. On the basis of optic symptoms, such as uveitis and serous retinal detachment in both eyes, increased cell counts in cerebrospinal fluid, and positivity for human leukocyte antigen (HLA)-DR4 in peripheral blood cells, incomplete VKH disease was diagnosed. Intravenous treatment with high-dose prednisolone (60mg/m2/day) for 7 days improved both visual acuity and serous retinal detachment and enabled the remains of the COPDAC chemotherapy cycle to be administered. With prednisolone treatment, visual acuity improved from 20/500 to 20/20 in the right eye and from 20/63 to 20/25 in the left eye. Because multiple vitiligo lesions later appeared in the abdomen, complete VKH disease was finally diagnosed. CONCLUSION: The onset of VKH disease occurred during chemotherapy for Hodgkin lymphoma. The patient was HLA-DR4-positive and might have had a predisposition to develop autoimmune diseases, including VKH disease. However, the anticancer drugs administered to this patient have not been reported to cause uveitis. Whether Hodgkin lymphoma triggered the development of VKH remains unclear. Early diagnosis of VKH disease and prompt treatment with high-dose prednisone enabled the patient to maintain good visual function despite chemotherapy for Hodgkin lymphoma.

EDI-OCT as an auxiliary examination for the differential diagnosis of bullous CSC and Vogt-Koyanagi-Harada: A case report.

A 46-year-old male patient visited our clinic with a complaint of blurred vision in the right eye accompanied by headache and insomnia. The fundus examination showed three bullous retinal detachments in the right eye. Considering the prodromal symptoms and other fundus characteristics such as vitreous cells in the posterior pole and multifocal fluorescence leakages on fundus fluorescein angiography (FFA), initial diagnosis was considered as Vogt-Koyanagi-Harada (VKH). However, oral glucocorticoids didn't improve patient's vision. Further enhanced depth imaging (EDI)-optical coherence tomography (OCT) scan displayed hyper-reflective lesions at the choroidal layer. We proposed that hyper-reflective lesions at the choroidal layer on EDI-OCT may characterize the bullous variant of central serous chorioretinopathy (CSC). After fundus photocoagulation treatment, the patient's vision improved.

Vogt-Koyanagi-Harada Disease: The Clinical Spectrum and Management of Case Series in a Tertiary Eye Centre in Northern Part Of Malaysia.

AIMS: We present the clinical spectrum, the initial clinical presentation with management trends in treating 14 Vogt-Koyanagi-Harada (VKH) disease cases in a tertiary center in the Northern part of Malaysia. CASE SERIES: There were 14 cases of Vogt-Koyanagi-Harada (VKH) disease retrospectively reviewed over five years (from 2015 to 2020). The mean age at presentation was 37.7 years (range 21-64 years), with female predominance (85.7%). All cases presented with acute uveitic stage and bilateral eye involvement. Of them, 11 (78.6%) were probable VKH, and three (21.4%) were incomplete VKH. All patients attended with acute panuveitis at first presentation. The main posterior segment involvement was disc edema in 57.1% (16 out of 28 eyes) and exudative retinal detachment (ERD) in 35.7% (10 out of 28 eyes). Most of them presented with blindness (3/60 and worse) and moderate visual impair- ment (6/18-6/60); 35.71% each, followed by mild visual impairment (6/12-6/18), and severe visual impairment (6/60-3/60); 7.1% each. Ten patients (71.4%) required combination second-line immunomodulatory treatment during subsequent visits, and only four patients (28.6%) responded well to corticosteroid therapy. Most of the cases achieved no visual impairment (64.3%), followed by mild visual impairment (21.4%) and moderate visual impairment (14.3%), and none were severe or blind at the end of follow-up. CONCLUSION: VKH is a potentially blinding illness if there is inadequate control of the disease in the acute stage. Most of our patients achieved good visual outcomes with early immunomodulatory treatment and systemic corticosteroids.

A Novel Risk Stratification-Based Immunomodulatory Treatment Strategy for Vogt-Koyanagi-Harada Disease.

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.

VKH with APECED in a Two-Year-Old Child: A Rare Concomitant Diagnosis in an Unprecedented Age.

PURPOSE: To present a case of Vogt Koyanagi Harada (VKH) associated with autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy syndrome (APECED) in a two-year-old. CASE PRESENTATION: A two-year-old type 1 diabetic with hypothyroidism presented with impaired fixation. Ocular examination revealed right vitritis, choroiditis, a hyperemic disc, and an area of exudative detachment. At the same time, there was no fundus view in the left eye, and ultrasonographic assessment revealed vitritis and a thickened choroid. Patient developed sunset glow fundus with alopecia, poliosis and vitiligo and a diagnosis of complete VKH with APECED was made. CONCLUSION: APECED is a rare endocrine disorder and has been reported to be associated with VKH twice. Likewise, VKH is commonly present in much older patients; this is the first time ever to be diagnosed in a two-year-old child.