Vogt-Koyanagi-Harada Research Articles

CLINICAL FEATURES AND PROGNOSTIC VALUE OF BACILLARY LAYER DETACHMENT IN ACUTE VOGT-KOYANAGI-HARADA DISEASE.

To evaluate the characteristics of bacillary layer detachment (BALAD) in acute Vogt-Koyanagi-Harada (VKH) disease and determine its prognostic value. Seventy patients with acute VKH disease with a minimum follow-up of 6 months were studied. The primary outcomes were clinical characteristics associated with BALAD, including features on multimodal imaging at baseline and follow-up. The secondary outcomes included best-corrected visual acuity and VKH with recurrence features. Of 70 eyes (36 patients), 41 (58.6%) showed BALAD. The mean baseline best-corrected visual acuity and mean best-corrected visual acuity after resolution of serous retinal detachment were significantly lower in the BALAD group than in the no-BALAD group (0.90 ± 0.49 vs. 0.35 ± 0.35 log minimum angle of resolution, P < 0.001 and 0.39 ± 0.27 vs. 0.20 ± 0.20 log minimum angle of resolution, P = 0.020). The loss of ellipsoid zone integrity at baseline, proportion of serous retinal detachment, duration of serous retinal detachment, loss of ellipsoid zone integrity at 1 month, and subfoveal choroidal thickness at baseline were significantly higher in the BALAD group ( P = 0.017, P = 0.006, P = 0.023, P = 0.002, and P = 0.046, respectively). The mean best-corrected visual acuity and subfoveal choroidal thickness did not differ between the two groups at 6 months ( P = 0.380 and P = 0.180, respectively). Bacillary layer detachment at baseline was found to be a significant prognostic factor for VKH with recurrence features ( P = 0.007). Vogt-Koyanagi-Harada with BALAD featured more severe clinical characteristics than VKH without BALAD during the acute phase. Patients with baseline BALAD require more vigilant monitoring as they are more likely to show recurrence features within the first 6 months.

Clinical features, diagnosis, and management of COVID-19 vaccine-associated Vogt-Koyanagi-Harada disease

ABSTRACT Vogt-Koyanagi-Harada (VKH) disease is a rare and serious ocular adverse reaction following COVID-19 vaccination. This study aimed to evaluate the clinical features, diagnosis and management of COVID-19 vaccine-associated VKH disease. Case reports of VKH disease after COVID-19 vaccination were collected up to February 11, 2023 for retrospective analysis. Twenty-one patients (9 males and 12 females) were included, with a median age of 45 years (range 19–78), from three main regions, Asia (12/21), the Mediterranean region (4/21), and South America (5/21). Fourteen patients developed symptoms after the first dose of the vaccine, and 8 after the second dose. Vaccines included mRNA vaccine (10 cases), virus vector vaccine (6 cases), and inactivated vaccine (5 cases). The average time interval from vaccination to onset of symptoms was 7.5 days (range 12 hours to 4 weeks). All 21 patients experienced visual impairment after vaccination, with 20 cases involving both eyes. Sixteen patients showed symptoms of meningitis. Serous retinal detachment was observed in 16 patients, choroidal thickening was observed in 14, aqueous cell in 9, and subretinal fluid in 6. CSF pleocytosis was detected in 7 patients and skin symptoms were found in 3 patients. All patients received corticosteroid therapy, and 8 also received immunosuppressive agents. All patients recovered well, with a mean recovery time of 2 months. Early diagnosis and early treatment are crucial to the prognosis of patients with VKH after vaccination with COVID-19 vaccine. The risk of vaccination against COVID-19 in patients with a history of VKH disease should be evaluated clinically.

A COVID-19 perspective of Vogt-Koyanagi-Harada disease.

Vogt-Koyanagi-Harada (VKH) disease, a bilateral granulomatous panuveitis associated with multisystem involvement, is a T-cell-mediated autoimmune disorder in which cytotoxic T-cell target melanocytes in genetically susceptible individuals. Recently, there has been an increase in literature on the new onset of uveitis and reactivation of previously diagnosed cases of uveitis following Covid-19 vaccinations. It has been postulated that Covid-19 vaccines can lead to an immunomodulatory change resulting in an autoimmune phenomenon in the recipients. VKH following COVID-19 infection was reported in four patients and a total of 46 patients developing VKH or VKH-like disease following COVID-19 vaccinations. There are reports of four patients who had been recovering or recovered from VKH after receiving the first dosage of the vaccine and developed worsening of ocular inflammation after receiving the second dose of the vaccine.

Does Subretinal Fluid Optical Density Ratio Differ Among the Eyes with Acute Central Serous Chorioretinopathy,Vogt Koyanagi Harada Disease and Choroidal Hemangioma: A Cross-sectional Study

PurposeTo investigate the diagnostic value of optical density ratio (ODR) in various diseases with subretinal fluid (SRF) due to different pathophysiologies. MethodsPatients with acute central serous chorioretinopathy, CSCR (n = 49), Vogt Koyanagi Harada disease, VKH (n = 34), and choroidal hemangioma (n = 17) characterized with SRF were included. Spectral-domain optical coherence tomography (SD-OCT) images were analyzed using ImageJ by three independent readers. The ODRs were calculated using “region of interest (ROI)” and “entire region (TOTAL)” selection methods from the SRF to the vitreous, retinal nerve fiber layer (RNFL), and retinal pigment epithelium (RPE) reflectivity ratios. A correlation analysis between age, central macular thickness (CMT), SRF height, SRF width, and ODRs were obtained. ResultsOptical density (OD) measurement was highly reproducible (intraclass correlation coefficient> 0.9). Optical density of the SRF, vitreous, RNFL, and signal strength were comparable (p = 0.360, p = 0.247, p = 0.105, and 0.628, respectively). There was no difference in SRF OD measurements between the two methods (p = 0.401), while there was a significant difference in vitreous OD measurements (p = 0.016). ANOVA test of ODR(ROI), ODR(TOTAL), ODR-RPE (ROI) and ODR-RNFL (ROI) revealed no significant difference among acute CSCR, VKH disease and choroidal hemangioma groups (p > 0.05 for all). Correlation analysis revealed a significant negative correlation between SRF height (p < 0.05) and CMT (p < 0.01) with SRF ODR(ROI). ConclusionODR measurement appears to be a highly repeatable SD-OCT parameter for diseases characterized with SRF collection. Despite variations in their pathophysiology, the ODR was not statistically different in acute CSCR, VKH disease, and choroidal hemangioma.

Association of ZC3HAV1 single nucleotide polymorphisms with the susceptibility of Vogt-Koyanagi-Harada Disease

BackgroundPolymorphisms of genes related to the immune response have been reported to confer susceptibility to Vogt-Koyanagi-Harada (VKH) disease. This study was carried out to determine whether zinc finger CCCH-type containing antiviral 1 (ZC3HAV1) and tripartite motif-containing protein 25 (TRIM25) genetic polymorphisms are associated with this disease.MethodsA total of 766 VKH patients and 909 healthy individuals were enrolled in this two-stage case-control study. Thirty-one tag single nucleotide polymorphisms (SNPs) of ZC3HAV1 and TRIM25 were genotyped by MassARRAY System and iPLEX Gold Genotyping Assay. Allele and genotype frequencies were analyzed by the χ2 test or Fisher’s exact test. Cochran-Mantel-Haenszel test was used to assess the pooled odds ratio (OR) in the combined study. A stratified analysis was performed in terms of the major clinical features of VKH disease.ResultsWe found a statistically significant increased frequency of the minor A allele of ZC3HAV1 rs7779972 (P = 1.50 × 10− 4, pooled OR = 1.332, 95%CI = 1.149–1.545) in VKH disease as compared with controls by using the Cochran-Mantel-Haenszel test. The GG genotype of rs7779972 showed a protective association with VKH disease (P = 1.88 × 10− 3, OR = 0.733, 95%CI = 0.602–0.892). There was no difference regarding the frequency of the remaining SNPs between VKH cases and controls (all P > 2.08 × 10− 3). The stratified analysis showed no significant association of rs7779972 with the major clinical characteristics of VKH disease.ConclusionOur study indicated that the ZC3HAV1 variant rs7779972 might confer susceptibility to VKH disease in Han Chinese.

The retinal bacillary layer detachment: Clinical features and outcomes in posterior uveitis

To describe the clinical characteristics, presentation and response to treatment in posterior uveitis patients with bacillary layer detachment (BLD) seen on optical coherence tomography (OCT). Retrospective review of patients with posterior uveitis and SD-OCT scans consistent with BLD. Data collected included demographics, uveitic etiology, treatment and duration of follow-up. Outcome measures included macular volume, central subfoveal thickness and visual acuity. Sixteen patients (20eyes) were included. Twelve were female (75%). The mean age was 43.68±14.7years. The most frequent etiology of the uveitis was Vogt-Koyanagi-Harada (VKH) disease (n=10), followed by sympathetic ophthalmia (n=2). BLD was bilateral in four patients. Eight patients were treated with intravenous methylprednisolone boluses. Immunosuppressive therapies were required in 8patients. The mean follow-up was 70months (range: 2.0-216.0). BLD was observed in a series of posterior uveitis cases of various etiologies, showing functional and structural resolution with treatment in most cases.

Acute phase clinical manifestations of patients with Vogt-Koyanagi-Harada disease in Southern China

BackgroundsTo characterize the acute phase clinical manifestations and visual outcomes of the patients with Vogt-Koyanagi Harada (VKH) disease in southern China.MethodsIn total, 186 patients with acute-onset VKH disease were recruited. The demographic data, clinical signs, ophthalmic examinations, and visual outcomes were analyzed.ResultsAmong the 186 VKH patients, 3 were diagnosed as complete VKH, 125 as incomplete VKH, and 58 as probable VKH. All patients visited the hospital within 3 months of onset and complained of decreased vision. For the extraocular manifestations, 121 patients (65%) referred neurological symptoms. Anterior chamber activity was negative in most eyes within an onset of 7 days, which increased slightly with onset beyond 1 week. Exudative retinal detachment (366 eyes, 98%) and optic disc hyperaemia (314 eyes, 84%) were commonly observed at presentation. A typical ancillary examination helped with the diagnosis of VKH. Systemic corticosteroid therapy was prescribed. The logMAR best-corrected visual acuity improved significantly from 0.74 ± 0.54 at baseline to 0.12 ± 0.24 at the 1-year follow-up visit. The recurrence rate was 18% in the follow-up visits. Erythrocyte sedimentation rate and C-reactive protein were significantly correlated to VKH recurrences.ConclusionPosterior uveitis, followed by mild anterior uveitis, is the typical initial manifestation in the acute phase of Chinese VKH patients. Visual outcome improvement is promising in most patients receiving systemic corticosteroid therapy in the acute phase. Detection of the clinical features at the initial onset of VKH could facilitate early treatment and better vision improvement.

Classification of Vogt-Koyanagi-Harada disease using feature selection and classification based on wide-field swept-source optical coherence tomography angiography.

Background: Vogt-Koyanagi-Harada (VKH) disease is a common and easily blinded uveitis entity, with choroid being the main involved site. Classification of VKH disease and its different stages is crucial because they differ in clinical manifestations and therapeutic interventions. Wide-field swept-source optical coherence tomography angiography (WSS-OCTA) provides the advantages of non-invasiveness, large-field-of-view, high resolution, and ease of measuring and calculating choroid, offering the potential feasibility of simplified VKH classification assessment based on WSS-OCTA. Methods: 15 healthy controls (HC), 13 acute-phase and 17 convalescent-phase VKH patients were included, undertaken WSS-OCTA examination with a scanning field of 15 × 9mm2. 20 WSS-OCTA parameters were then extracted from WSS-OCTA images. To classify HC and VKH patients in acute and convalescent phases, two 2-class VKH datasets (HC and VKH) and two 3-class VKH datasets (HC, acute-phase VKH, and convalescent-phase VKH) were established by the WSS-OCTA parameters alone or in combination with best-corrected visual acuity (logMAR BCVA) and intraocular pressure (IOP), respectively. A new feature selection and classification method that combines an equilibrium optimizer and a support vector machine (called SVM-EO) was adopted to select classification-sensitive parameters among the massive datasets and to achieve outstanding classification performance. The interpretability of the VKH classification models was demonstrated based on SHapley Additive exPlanations (SHAP). Results: Based on pure WSS-OCTA parameters, we achieved classification accuracies of 91.61% ± 12.17% and 86.69% ± 8.30% for 2- and 3-class VKH classification tasks. By combining the WSS-OCTA parameters and logMAR BCVA, we achieved better classification performance of 98.82% ± 2.63% and 96.16% ± 5.88%, respectively. Through SHAP analysis, we found that logMAR BCVA and vascular perfusion density (VPD) calculated from the whole field of view region in the choriocapillaris (whole FOV CC-VPD) were the most important features for VKH classification in our models. Conclusion: We achieved excellent VKH classification performance based on a non-invasive WSS-OCTA examination, which provides the possibility for future clinical VKH classification with high sensitivity and specificity.

Impact of Initial Management on Disease Evolution in Vogt–Koyanagi–Harada Syndrome: A Retrospective Cohort of 50 Patients

ABSTRACT Purpose The study aims to determine the impact of initial management in Vogt–Koyanagi–Harada syndrome (VKHS). Methods Patients diagnosed with a VKHS between January 2001 and December 2020 in two French tertiary centers were included in a retrospective study. Results Fifty patients were included with a median duration of follow-up of 29.8 months. All patients received oral prednisone after methylprednisolone in all but four of them. Five patients received at least one associated immunosuppressive therapy (IST) within the first 6 months and 26 patients received IST during the entire follow-up period. Twenty-eight patients presented at least one relapse at a median of 5.4 months from diagnosis. Multivariate analyses demonstrated a significant association between relapse and delayed treatment (>26 days) (HR = 3.69, CI95% 1.30–10.47, p = .01), whereas no association was observed between relapse and the number of corticosteroid pulses at initial management. Conclusion An early corticosteroid treatment within the first 26 days of symptoms decreased the relapse rate.

The Role of Widefield Optical Coherence Tomography Angiography in the Diagnosis and Management of Acute Vogt–Koyanagi–Harada Disease

ABSTRACT Purpose We explore the choroid vasculature changes of acute Vogt–Koyanagi–Harada (VKH) disease using widefield optical coherence tomography angiography (OCTA). Methods In this retrospective, observational, longitudinal study, 16 patients with acute VKH disease (32 eyes, mean age: 42.19 ±13.66 years) were measured using widefield OCTA. Results In this study, we first described the multiple dark foci in choriocapillaris and Sattler’s layer in the panoramic montage of the five 12x12mm images in 30 eyes (93.8%) of acute VKH disease. OCTA follow-up in these 30 eyes demonstrated the diminished size and number of these dark foci in choriocapillaris and Sattler’s layer after the initiation of treatment. Conclusions Widefield OCTA enables noninvasive identification of characteristics of flow void at the level of superficial choroidal vessels in the acute phase and may be a novel valuable tool for diagnosis and monitoring of disease progression in VKH disease in the future.

TET2-mediated upregulation of 5-hydroxymethylcytosine in LRRC39 promoter promotes Th1 response in association with downregulated Treg response in Vogt-Koyanagi-Harada disease

DNA 5-Hydroxymethylcytosine (5-hmC), an oxidative reaction mediated by the ten-eleven translocation (TET) family, has been reported to play an essential role in the progression of auto-inflammatory and autoimmune diseases. By far, little is known about the effect of DNA 5-hmC and the TET family on the development of Vogt–Koyanagi–Harada (VKH) disease. In this study, we discovered that the global DNA 5-hmC level and the TET activity were elevated in association with the up-regulated expression of TET2 at both mRNA and protein levels in CD4+T cells from active VKH patients compared to healthy controls. Integrated analysis of DNA 5-hmC pattern and transcription profile of CD4+ T cells revealed that 6 candidate target genes were involved in the development of VKH disease. The promoter 5-hmC and mRNA levels of leucine rich repeat containing 39 (LRRC39) were verified to be elevated in active VKH patients. Functional experiments showed that TET2 could up-regulate LRRC39 mRNA expression by increasing the promoter 5-hmC level of LRRC39 in CD4+ T cells from active VKH patients. Up-regulated LRRC39 expression could increase the frequencies of IFN-γ+ and IL-17+ CD4+ T cells as well as the secretions of IFN-γ and IL-17 in association with the decreased frequency of CD4+CD25+FOXP3+ regulatory T (Treg) cells and the reduced production of IL-10. Additionally, restoration of LRRC39 rescued TET2-silencing-mediated reduced frequency of IFN-γ+ CD4+ T cells and increased frequency of CD4+CD25+FOXP3+ Treg cells. Collectively, our study reveals a novel axis, the TET2–5-hmC-LRRC39–Th1/Treg responses axis, in the pathogenesis of VKH and provides a potential target for further investigation into the epigenetic therapy of this disease.

Successive onset of Vogt-Koyanagi-Harada syndrome in father and son

BackgroundVogt‒Koyanagi‒Harada (VKH) disease is a multifactorial systemic autoimmune disorder against melanocytes that is characterized by panuveitis. Familial occurrence of VKH disease is rare. Here, we report two cases of a father and his son with characteristic manifestations of VKH disease.Case presentationA 53-year-old male with typical clinical symptoms of VKH disease was referred to Tangshan Eye Hospital. Examination showed the presence of ciliochoroidal effusion and exudative retinal detachment in both eyes. The patient was given intravenous methylprednisolone 120 mg for 2 days and intravenous methylprednisolone 80 mg for 1 day followed by 48 mg (1 mg/kg/day) oral methylprednisolone daily, accompanied by oral azathioprine 50 mg daily. Cycloplegic agent (0.5% tropicamide three times daily [TID]) was added. The patient was free of symptoms and recurrence within more than 1-year-follow-up period, the best corrected visual acuity (BVCA) was increased and maintained in both eyes with complete resolution of subretinal fluid. One year and nine months later, case 2 (his son) also presented with the typical clinical symptoms of VKH disease at 29 years of age. The son also recovered from VKH disease after routine and standard treatment.ConclusionsTo the best of our knowledge, this is the first VKH disease case report of a father-son relationship. Although genetic factors have been demonstrated to be involved in the pathogenesis of VKH disease, the different inheritance modes of VKH patients need to be further explored and studied.

Retinal Pigment Epithelial Characteristics in Acute and Resolved Vogt-Koyanagi-Harada Disease

Vogt-Koyanagi-Harada (VKH) disease is an auto-immune inflammatory disease of choroidal origin. During the acute stage, optical coherence tomography (OCT), however, may not be able to assess the entire choroid. The aims of the paper were to evaluate the role of retinal pigment epithelium (RPE) as a biomarker of inflammation in acute VKH. This was a retrospective observational study done in 55 eyes of 29 patients with acute VKH. RPE thickness, total choroidal thickness, and RPE reflectivity before and after resolution were analyzed using image J software. Correlations between several baseline and post-resolution parameters were performed, and factors affecting change in visual acuity were analyzed. A significant decrease in RPE thickness and a significant increase in RPE reflectivity were seen following resolution of the disease. Furthermore, there was a significant correlation between RPE and choroidal thickness during the acute stage of the disease. Baseline visual acuity and the presence of bacillary detachment at baseline were the only factors responsible for changes in visual acuity. We propose the utility of RPE layer as a surrogate biomarker of choroidal activity and inflammation in terms of RPE reflectivity and RPE thickness during the acute stage of VKH, especially when there is poor imaging of the choroid.

Incidence and pre/post-treatment risk factors of glaucoma in Vogt-Koyanagi-Harada disease.

To investigate the incidence and pre/post-treatment risk factors of glaucoma in patients with Vogt-Koyanagi-Harada (VKH) disease. Data regarding secondary glaucoma were collected from the medical records of patients with VKH disease who were followed up at the uveitis service at Hiroshima University for more than 6months. We examined the incidence of glaucoma and pre/post-treatment risk factors for glaucoma in patients with VKH disease. Forty-nine patients with VKH disease were included in this study (31 women and 18 men). The mean age at onset was 50.4 ± 15.4years and the mean length of follow-up was 40.7 ± 25.5months. The most common initial treatment was pulse intravenous corticosteroid therapy (89.8%). Fifteen patients developed secondary glaucoma during follow-up. The median time of glaucoma onset from VKH development was 4.5months (range 0-44months). Disc swelling type as a pre-treatment factor (p = 0.089, hazard ratio = 7.268), worse final best corrected visual acuity (p = 0.099, odds ratio = 1.545), and cataract progression (p = 0.076, odds ratio = 7.886) as post-treatment factors showed trends for glaucoma development. The patients who progressed to the chronic recurrent stage had more complications including glaucoma. Secondary glaucoma occurred in more than 30% of patients with VKH disease. The factors that showed a trend toward glaucoma development may reflect an association with delayed treatment initiation and prolonged ocular inflammation.

Case Reports of Children with Vogt_Koyanagi_Harada Syndrome

Purpose: To assess the symptoms of Vogt-Koyanagi-Harada (VKH) syndrome in Moroccan children. Methods: Clinical data were acquired from the medical records of three children with VKH disease at the Marrakech University Children's Hospital. Results: Three cases fulfilled the VKH diagnostic criteria. The patients, ranging in age from 4 to 12 years, all had chronic illness. Uveitis was the most common ocular finding. Glaucoma, cataract, posterior synechiae, and subretinal neovascularization were complications for the patients. Conclusion: Depending on the long- term consequences, visual results were positive in all of the patients. VKH is uncommon in children, but it can be sight- threatening and requires close monitoring, making it an essential differential diagnosis.

Vogt-Koyanagi-Harada-like Syndrome and Electronegative Electroretinogram after Nivolumab Therapy for Metastatic Cutaneous Melanoma: A Case Report

Background: Vogt-Koyanagi-Harada-like (VKH) syndrome or electronegative electroretinograms (ERG) have both been described before or after immunotherapy for metastatic cutaneous melanoma, but they have not been described occurring together. Objective: The objective of this study is to describe a case of VKH-like syndrome occurring simultaneously with an electronegative ERG after nivolumab therapy for metastatic cutaneous melanoma. Methods: Case report of a patient with ocular findings after nivolumab therapy for metastatic melanoma was reported. Multimodal imaging, including color fundus photography, optical coherence tomography (OCT), and both full-field and multifocal ERGs were used to describe the findings. Literature review was conducted with PubMed. Results: We reported a case of a patient with nivolumab-treated melanoma presenting with presumed VKH-like syndrome with panuveitis, choroidal depigmentation, and cutaneous vitiligo, as well as melanoma-associated retinopathy (MAR)-like electronegative ERG findings. Nivolumab was stopped and corticosteroid therapy was initiated. Although the patient’s visual acuity remained severely limited, her inflammation resolved, and the areas of choroidal depigmentation slowly decreased over years of subsequent follow-up. Conclusion: Vision loss accompanied by simultaneous VKH-like findings with choroidal vitiligo and a MAR-like electronegative ERG may develop after nivolumab therapy. The uveitic and vitiligo may improve with immunosuppressive therapy, but the vision loss and ERG findings may persist.

PROBABLE VOGT-KOYANAGI-HARADA DISEASE: A CASE REPORT

Vogt-Koyanagi-Harada(VKH)diseaseisabilateralgranulomatouspanuveitis affecting young adults, withor without systemic manifestations. The prognosis of VKH disease is generally favorable, however the development of complicationscanleadto blindnessanddeafness.(1) In this case, we report a 22-year-old man who developed probable Vogt–Koyanagi–Haradadisease without any systemic symptoms, but with several risk factors for poor visual prognosisbeforetreatment,andwhowassuccessfullytreatedwithintensivecorticosteroidtherapy. Our aim is to describe a clinical case with bilateral serous retinal detachment that presumed asprobableVKHdisease.

Vogt-Koyanagi-Harada Syndrome Diagnosed at the Sequellar Stage in an 11-Year-Old Child: A Case Report

Vogt-Koyanagi-Harada syndrome is a severe granulomatous panuveitis associated with neuromeningeal, dermatologic, and auditory extraocular manifestations. Uveitis in children accounts for only 5-10% of all uveitis. Vogt-Koyanagi-Harada syndrome is an even rarer cause. The exact etiology remains unknown, however, a genetic predisposition to an autoimmune process directed against melanocytes is incriminated. We report the case of an 11-year-old child with a history of recurrent red eyes with episodes of headache accompanied by dizziness who consulted us for a painful red eye with decreased visual acuity. In view of the clinical and paraclinical anamnestic elements and based on the diagnostic criteria of the Vogt-Koyanagi-Harada syndrome of the American Uveitis Society revised in 2001, the diagnosis of a VKH syndrome in its complete form was retained. Treatment with 3 boluses of methylprednisolone (1 g/1.73m2) over three days was initiated, followed by oral therapy combined with topical corticosteroids and cycloplegic therapy. The long-term evolution was marked by recurrences of the previous uveitis, and the patient was put on immunosuppressive treatment with mycophenolate mofetil (30 mg/kg per day in two doses). Although rare, VKH syndrome exists in the pediatric population. Our observation highlights the diagnostic difficulties associated with this condition. We insist on the usefulness of a thorough history in order to retrace the history of the disease and on the necessity of an early diagnosis and treatment to guarantee a better prognosis.

Genetic association of PRKCD and CARD9 polymorphisms with Vogt–Koyanagi–Harada disease in the Chinese Han population

BackgroundProtein kinase C delta (PRKCD) and caspase recruitment domain family member 9 (CARD9) are genes involved in B and T cell activation, and cytokine production, which are vital mechanisms underlying autoimmune disease development. This study aimed to explore the association of the PRKCD and CARD9 genes with Vogt–Koyanagi–Harada disease (VKH) disease. The case–control study was performed to in 912 patients with VKH and 878 normal controls. MassARRAY system, SHEsis online platform, real-time PCR, and enzyme-linked immunosorbent assay were used to detect genotyping, haplotyping, mRNA expression, and cytokine levels, respectively.ResultsWe found that rs74437127 C allele of PRKCD, rs3812555 CC genotype, and C allele of CARD9 were associated with increased susceptibility of VKH (Pc = 0.020, OR = 1.624; Pc = 2.04 × 10–5, OR = 1.810; Pc = 2.76 × 10–5, OR = 1.698, respectively). However, the rs74437127 T allele, and rs3812555 TC genotype and T allele were linked with decreased susceptibility to VKH (Pc = 0.020, OR = 0.616; Pc = 7.85 × 10–5, OR = 0.559; Pc = 2.76 × 10–5, OR = 0.589, respectively). PRKCD ATG and CARD9 GCTTA haplotypes decreased susceptibility to VKH (Pc = 3.11 × 10–3, OR = 0.594; Pc = 5.00 × 10–3, OR = 0.639, respectively). Functional studies on rs3812555 genotyped individuals revealed that CC carriers had significantly higher CARD9 mRNA expression and tumour necrosis factor-α production than TC/TT carriers (P = 1.00 × 10–4; P = 2.00 × 10–3, respectively).ConclusionsWe found an association between PRKCD rs74437127 and CARD9 rs3812555 polymorphisms and VKH susceptibility and revealed that the increased susceptibility of rs3812555 for VKH may be mediated by regulating CARD9 gene expression and the production of pro-inflammatory cytokines, such as TNF-α.

Vogt-Koyanagi-Harada disease following ChAdOx1 nCoV-19 and mRNA-1273 vaccination

Almost all vaccines have been reported to be associated with ocular inflammation, which has caused some concern regarding global mass COVID-19 vaccination efforts. Vogt-Koyanagi-Harada disease (VKHD) is a granulomatous inflammation caused by an autoimmune response against antigens in melanocytes, including those in the eyes. The mechanism by which COVID-19 vaccines are associated with VKHD is still unclear. Here, we report two cases of VKHD following COVID-19 vaccination. The first is a case of probable VKHD that presented with bilateral vision loss after administration of the adenovirus-vectored vaccine ChAdOx1 nCoV-19 (AstraZeneca). The condition improved after intravenous methylprednisolone 1g daily for 3days, followed by oral methotrexate and a slow taper of oral corticosteroids. The second case is a patient with an established diagnosis of well-controlled VKHD who developed a reactivation of the disease after receiving the mRNA-based vaccine (mRNA-1273, Moderna). VKHD is a potential ocular event that could follow COVID-19 vaccination. Awareness of this association is key to early detection and treatment to prevent loss of vision.