Vogt Koyanagi Harada

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...

A 55-year-old woman with dyspnea was diagnosed with a 9.5cm left renal clear cell carcinoma and extensive metastatic disease. Initial treatment with Sunitinib was effective but discontinued due to severe dermatitis. Nivolumab therapy led to complete metastasis resolution and consequently nephrectomy was performed at 12 months. Postoperatively, she developed Vogt-Koyanagi-Harada-like disease, necessitating Nivolumab suspension and vision improvement with corticosteroids. After 24 disease-free months, a new contralateral renal lesion and pulmonary metastases were identified, prompting cabozantinib treatment...

This review aims to provide an understanding of the diagnostic and therapeutic challenges of uveitis associated with immune checkpoint inhibitors (ICI). In the wake of these molecules being increasingly employed as a treatment against different cancers, cases of uveitis post-ICI therapy have also been increasingly reported in the literature, warranting an extensive exploration of the clinical presentations, risk factors, and pathophysiological mechanisms of ICI-induced uveitis. This review further provides an understanding of the association between ICIs and uveitis, and assesses the efficacy of current diagnostic tools, underscoring the need for advanced techniques to enable early detection and accurate assessment...

PURPOSE: To investigate for association between skin autofluorescence (SAF) advanced glycation end products (AGEs) and uveitis/scleritis activity in systemic inflammatory disease-related active non-infectious uveitis/scleritis patients. METHODS: This cross-sectional study was conducted at Siriraj Hospital during October 2019 to March 2020. AGEs were measured by SAF method in systemic immune-related disease patients with active uveitis/scleritis, and those results were compared with those of healthy age-matched controls...

PURPOSE: To describe disease characteristics and outcomes of Vogt-Koyanagi-Harada (VKH) disease in elderly patients. METHODS: Retrospective analysis of patients older than 50 years with VKH disease at two referral centers in India. Demographics, extraocular and ocular involvement, treatment, complications, and visual acuity outcomes were noted. RESULTS: In total, 69 patients (mean age at presentation: 56.4 ± 4.7 years) were analyzed; 6/69 patients had diabetes mellitus at presentation, and 10/69 had hypertension...

Vogt-Koyanagi-Harada (VKH) disease and Behcet's uveitis (BU) are the two major vision-threatening uveitis entities. This study performed the first label-free quantitative proteomics on aqueous humor-derived exosomes from 84 patients with VKH or BU to determine their potential roles. Sixty-five differentially expressed proteins (DEPs) and 40 DEPs were detected in the VKH and BU groups, respectively. GO and KEGG analysis showed that DEPs were mainly enriched in the complement-related pathways. The complement C1q subcomponent subunit B (C1QB) was identified as a key exosomal protein, and its expression was significantly increased by western blotting in both diseases...

We report a case of Vogt-Koyanagi-Harada (VKH) disease that recurred 46 years after initial treatment. A 59-year-old woman presented with a 2-month history of bilateral vision blurring. She had received her third dose of coronavirus disease 2019 (COVID-19) vaccination 4 months before the onset of blurring. The best-corrected visual acuity (BCVA) was 1.0 in the right eye and 0.15 in the left eye at the initial visit. Iritis and synechia were observed between the lens and iris bilaterally. A sunset glow fundus was found in both eyes with no serous retinal detachments or disc hyperemia...

Vogt-Koyanagi-Harada (VKH) disease is a severe autoimmune disease. Herein, whole-exome sequencing (WES) study are performed on 2,573 controls and 229 VKH patients with follow-up next-generation sequencing (NGS) in a collection of 2,380 controls and 2,278 VKH patients. A rare c.188T>C (p Val63Ala) variant in the olfactory receptor 11H1 (OR11H1) gene is found to be significantly associated with VKH disease (rs71235604, Pcombined = 7.83 × 10-30 , odds ratio = 3.12). Functional study showes that OR11H1-A63 significantly increased inflammatory factors production and exacerbated barrier function damage...

Autoimmune uveitis (AU) is a severe disorder causing poor vision and blindness. However, the cellular dynamics and pathogenic mechanisms underlying retinal injury in uveitis remain unclear. In this study, single-cell RNA sequencing of the retina and cervical draining lymph nodes in experimental autoimmune uveitis mice was conducted to identify the cellular spatiotemporal dynamics and upregulation of the glycolysis-related gene LDHA. Suppression of LDHA can rescue the imbalance of T effector (Teff) cells/T regulator (Treg) cells under inflammation via downregulation of the glycolysis-PI3K signaling circuit and inhibition of the migration of CXCR4+ Teff cells towards retinal tissue...

BACKGROUND: To present a rare case of a bilateral immune checkpoint inhibitor- (ICI-) induced photoreceptor injury with a bacillary layer detachment (BALAD) and a dissection of the photoreceptor inner and outer segment, accompanied by ICI-induced Vogt-Koyanagi-Harada- (VKH-) like uveitis after initial administration of nivolumab and ipilimumab. Case Presentation . A 52-year-old female with metastatic malignant cutaneous melanoma experiencing bilateral progressive visual acuity reduction, after treatment initiation with 1 mg/kg nivolumab and 3 mg/kg ipilimumab two weeks prior symptom onset...

PURPOSE: To report clinical profiles of multiple sclerosis (MS)-associated uveitis in seven cases from a single tertiary eye care center in South India. METHODS: Retrospective case series 2013-2023. RESULTS: Seven cases of MS-associated uveitis were retrieved from our databases. There were five females and two males. The diagnosis of MS was made by the neurologist in all cases. Bilaterality was seen in all cases. Intermediate uveitis was the most common presentation (five cases)...

Vogt-Koyanagi-Harada disease (VKH) is an autoimmune disease, and glucocorticoid therapy (GC) is widely used for VKH. We provided a profile of leukocyte populations and serum cytokines in VKH patients under GC. A prospective observational study was conducted on three treatment-naïve VKH patients. Peripheral blood samples were collected from the patients before GC (VKH-acute) and after 6 months (VKH-remission), and healthy individuals were used as controls. Proportions of 37-type leukocytes and levels of 27-kind cytokines were measured by mass cytometry and multiplex bead analysis...

AIM: The aim of this research is to evaluate the demographic attributes and clinical manifestations of uveitis in adult patients frequenting the Mansoura Ophthalmic Center. METHODS: Utilizing a cross-sectional, prospective, analytical study design, this research engaged adult patients visiting the outpatient uveitis clinic at the Mansoura Ophthalmic Center. Comprehensive case evaluations involved collecting detailed patient histories, examining ophthalmic records, and conducting thorough ocular examinations...

PURPOSE: To investigate the prognostic value of pretreatment indocyanine green angiographic (ICGA) features in initial-onset acute uveitis associated with Vogt-Koyanagi-Harada (VKH) disease. METHODS: Retrospective analysis of 84 patients (168 eyes). Main outcome measures were final visual acuity, development of 'sunset glow fundus' (SGF) and progression to chronic recurrent evolution. RESULTS: Thirty-eight patients (76 eyes) presented in the phase preceding anterior segment (AS) inflammation (early presentation) and 46 patients (92 eyes) had AS inflammation at presentation (late presentation)...

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...

The combination of BRAF kinase inhibitors (BRAFis) and MEK kinase inhibitors (MEKis) is one of the most promising chemotherapy regimens in the treatment of BRAF-mutant melanoma. Although BRAFi plus MEKi combined therapy is widely used for the treatment of BRAFV600 -mutated melanoma, the incidence of uveitis caused by BRAFi plus MEKi is limited. In this report, we described five cases (two men and three women) of Vogt-Koyanagi-Harada (VKH) disease-like uveitis in melanoma patients who received BRAFi plus MEKi combined therapy...

PURPOSE: Determining uveitis etiology is a challenge. It is based primarily on demographic data and the characteristics of eye examination. It is not clear to what extent extraocular physical signs contribute to elucidating the etiology. This study aimed to establish the contribution of the clinical extra-ophthalmological features for the assessment of the underlying etiology of uveitis. METHODS: We retrospectively reviewed 1307 patients with uveitis referred to our tertiary center between 2003 and 2021...

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...

BACKGROUND: To evaluate the most common causes of optic disc edema (ODE) in patients with significantly compromised vision (initial best-corrected visual acuity [BCVA] of 20/400 or worse) at presentation. METHODS: Retrospective chart review over a 5-year period of consecutive patients presenting to tertiary neuro-ophthalmology clinics at the University of Toronto. RESULTS: A total of 656 patients with ODE were included, and 49 patients (7...

The Vogt-Koyanagi-Harada syndrome (VKHS) is a unique form of granulomatous autoimmune disease that mostly impacts the pigmented tissues of the body. The main feature is bilateral granulomatous panuveitis, which is detected on ophthalmologic examination, along with additional systemic signs such as vitiligo, white hair, neurological involvement, or hearing loss. This study aims to report two cases of Vogt-Koyanagi-Harada syndrome presented in the children age group, which is unusual and very rare, to improve recognition of this disease to avoid complications and delay referral...