Vogt-Koyanagi-Harada Research Articles

Visual outcomes and choroidal thickness associated with human leukocyte antigen DRB1*04 in unclassifiable uveitis in Japanese patients

PurposeHuman leukocyte antigen (HLA) and immunity are related. Uveitis is also closely related to immunity. For example, the common presence of human leukocyte antigen (HLA)-DRB1*04 in the immune response is well known. The aim of this study was to investigate the relationship between visual prognosis and various HLA alleles before and after therapy in patients with unclassifiable uveitis, excluding those with Vogt-Koyanagi-Harada (VKH) disease.MethodsThis retrospective case series included 42 eyes from 22 consecutive patients with unclassifiable uveitis, excluding those with VKH disease. Visual acuity (VA), sex, refractive error, central retinal thickness (CRT), central choroidal thickness (CCT), and duration from onset to treatment were measured at initial and 6-month visits. Mean values of parameters were compared at each visit. Genotyping was performed by polymerase chain reaction amplification with sequence-specific primers.ResultsDRB1*04 showed a dominant change. No significant difference was observed in the other alleles. In DRB1*04, The mean differences in initial CCT, 6-month CCT, and 6-month VA showed statistically significant difference was found in best-corrected visual acuity (BCVA) between DRB1*04+ and DRB1*04− at the first visit. BCVA values at baseline and at the final visit were 0.13 ± 0.29 and 0.20 ± 0.36 in the DRB1*04+ and 0.00045 ± 0.20 and − 0.058 ± 0.11 in the DRB1*04− groups(p = 0.00465). Central Choroidal Thickness (CCT) values pretreatment and at the final visit after treatment were (pretreatment:361.00 ± 361.0 μm,after treatment: 286.00 ± 106.53 μm, p = 0.0174) in the DRB1*04+ group, and (pretreatment:281.3 ± 139.68 μm,after treatment:223.85 ± 99.034 μm, p = 0.0426) in the DRB1*04− group, respectively, indicating changes between baseline and the final visit. CCT was significantly greater in the DRB1*04+ group at both the initial visit and at 6 months. Multivariate analysis showed a significant difference between the presence or absence of DRB1*04 and sex.ConclusionHLA-DRB1*04 allele may affect visual prognosis and CCT in unclassifiable uveitis.

Prevalence, risk factors and management of ocular hypertension or glaucoma in patients with Vogt-Koyanagi-Harada disease

Background/aimsThis study was performed to examine the prevalence, risk factors and treatment outcome of OHT/glaucoma in Chinese patients with Vogt-Koyanagi-Harada (VKH).MethodsRetrospective non-interventional case series were conducted on a total of...

Epigenetic Modifications and Therapy in Uveitis

Uveitis is a sight-threatening intraocular inflammation, and the exact pathogenesis of uveitis is not yet clear. Recent studies, including multiple genome-wide association studies (GWASs), have identified genetic variations associated with the onset and progression of different types of uveitis, such as Vogt–Koyanagi–Harada (VKH) disease and Behcet’s disease (BD). However, epigenetic regulation has been shown to play key roles in the immunoregulation of uveitis, and epigenetic therapies are promising treatments for intraocular inflammation. In this review, we summarize recent advances in identifying epigenetic programs that cooperate with the physiology of intraocular immune responses and the pathology of intraocular inflammation. These attempts to understand the epigenetic mechanisms of uveitis may provide hope for the future development of epigenetic therapies for these devastating intraocular inflammatory conditions.

Mucosal-associated invariant T cells have therapeutic potential against ocular autoimmunity.

Autoimmune uveitis is a sight-threatening disease induced by pathogenic T cells that recognize retinal antigens; it is observed in disorders including Vogt-Koyanagi-Harada disease (VKH). The roles of specific T cell subsets and their therapeutic potential against autoimmune uveitis are not fully understood. Here we conducted multi-parametric single-cell protein quantification which shows that the frequency of CD161highTRAV1-2+ mucosal-associated invariant T (MAIT) cells that recognize vitamin B2 metabolite-based antigens is decreased in relapsing VKH patients compared to individuals without active ocular inflammation. An experimental autoimmune uveitis (EAU) mouse model revealed that genetic depletion of MAIT cells reduced the expression of interleukin (Il) 22 and exacerbated retinal pathology. Reduced IL-22 levels were commonly observed in patients with relapsing VKH compared to individuals without active ocular inflammation. Both mouse and human MAIT cells produced IL-22 upon stimulation with their antigenic metabolite in vitro. An intravitreal administration of the antigenic metabolite into EAU mice induced retinal MAIT cell expansion and enhanced the expressions of Il22, as well as its downstream genes related to anti-inflammatory and neuroprotective effects, leading to an improvement in both retinal pathology and visual function. Taken together, we demonstrate that a metabolite-driven approach targeting MAIT cells has therapeutic potential against autoimmune uveitis.

New Perspectives on the Immunopathogenesis and Treatment of Uveitis Associated With Vogt-Koyanagi-Harada Disease.

Uveitis associated with Vogt-Koyanagi-Harada (VKH) disease is a bilateral, chronic, granulomatous autoimmune disease associated with vitiligo, poliosis, alopecia, and meningeal and auditory manifestations. The disease affects pigmented races with a predisposing genetic background. Evidence has been provided that the clinical manifestations are caused by a T-lymphocyte-mediated autoimmune response directed against antigens associated with melanocytes in the target organs. Alongside of T lymphocytes, autoreactive B cells play a central role in the development and propagation of several autoimmune diseases. The potential role of B lymphocytes in the pathogenesis of granulomatous uveitis associated with VKH disease is exemplified within several studies. The early initial-onset acute uveitic phase typically exhibits granulomatous choroiditis with secondary exudative retinal detachment and optic disc hyperemia and swelling, subsequently involving the anterior segment if not adequately treated. The disease eventually progresses to chronic recurrent granulomatous anterior uveitis with progressive posterior segment depigmentation resulting in “sunset glow fundus” appearance and chorioretinal atrophy if not properly controlled. Chronically evolving disease is more refractory to treatment and, consequently, vision-threatening complications have been recognized to occur in the chronic recurrent phase of the disease. Conventional treatment with early high-dose systemic corticosteroids is not sufficient to prevent chronic evolution. Addition of immunomodulatory therapy with mycophenolate mofetil as first-line therapy combined with systemic corticosteroids in patients with acute initial-onset disease prevents progression to chronic evolution, late complications, vitiligo, and poliosis. Furthermore, patients under such combined therapy were able to discontinue treatment without relapse of inflammation. These findings suggest that there is a therapeutic window of opportunity for highly successful treatment during the early initial-onset acute uveitic phases, likely because the underlying disease process is not fully matured. It is hypothesized that early and aggressive immunosuppressive therapy will prevent remnant epitope generation in the initiation of the autoimmune process, the so-called primary response. B cell depleting therapy with the anti-CD20 monoclonal antibody rituximab is effective in patients with refractory chronic recurrent granulomatous uveitis. The good response after rituximab therapy reinforces the idea of an important role of B cells in the pathogenesis or progression of chronic recurrent uveitis associated with VKH disease.

Calculation of Similarity Between 26 Autoimmune Diseases Based on Three Measurements Including Network, Function, and Semantics.

Autoimmune diseases (ADs) are a broad range of diseases in which the immune response to self-antigens causes damage or disorder of tissues, and the genetic susceptibility is regarded as the key etiology of ADs. Accumulating evidence has suggested that there are certain commonalities among different ADs. However, the theoretical research about similarity between ADs is still limited. In this work, we first computed the genetic similarity between 26 ADs based on three measurements: network similarity (NetSim), functional similarity (FunSim), and semantic similarity (SemSim), and systematically identified three significant pairs of similar ADs: rheumatoid arthritis (RA) and systemic lupus erythematosus (SLE), myasthenia gravis (MG) and autoimmune thyroiditis (AIT), and autoimmune polyendocrinopathies (AP) and uveomeningoencephalitic syndrome (Vogt-Koyanagi-Harada syndrome, VKH). Then we investigated the gene ontology terms and pathways enriched by the three significant AD pairs through functional analysis. By the cluster analysis on the similarity matrix of 26 ADs, we embedded the three significant AD pairs in three different disease clusters respectively, and the ADs of each disease cluster might have high genetic similarity. We also detected the risk genes in common among the ADs which belonged to the same disease cluster. Overall, our findings will provide significant insight in the commonalities of different ADs in genetics, and contribute to the discovery of novel biomarkers and the development of new therapeutic methods for ADs.

Overexpressing Kallistatin Aggravates Experimental Autoimmune Uveitis Through Promoting Th17 Differentiation.

Kallistatin or kallikrein-binding protein (KBP) has been reported to regulate angiogenesis, inflammation and tumor progression. Autoimmune uveitis is a common, sight-threatening inflammatory intraocular disease. However, the roles of kallistatin in autoimmunity and autoreactive T cells are poorly investigated. Compared to non-uveitis controls, we found that plasma levels of kallistatin were significantly upregulated in patients with Vogt-Koyanagi-Harada (VKH) disease, one of the non-infectious uveitis. Using an experimental autoimmune uveitis (EAU) model induced by human interphotoreceptor retinoid-binding protein peptide 651-670 (hIRBP651-670), we examined the effects of kallistatin on the pathogenesis of autoimmune diseases. Compared to wild type (WT) mice, kallistatin transgenic (KS) mice developed severe uveitis with dominant Th17 infiltrates in the eye. In addition, the proliferative antigen-specific T cells isolated from KS EAU mice produced increased levels of IL-17A, but not IFN-γ or IL-10 cytokines. Moreover, splenic CD4+ T cells from naïve KS mice expressed higher levels of Il17a mRNA compared to WT naïve mice. Under Th17 polarization conditions, KS mice exhibited enhanced differentiation of naïve CD4+ T cells into Th17 cells compared to WT controls. Together, our results indicate that kallistatin promotes Th17 differentiation and is a key regulator of aggravating autoinflammation in EAU. Targeting kallistatin might be a potential to treat autoimmune disease.

Microvasculature Features of Vogt-Koyanagi-Harada Disease Revealed by Widefield Swept-Source Optical Coherence Tomography Angiography.

Background: Vogt-Koyanagi-Harada (VKH) disease is a multisystem autoimmune disorder which could induce bilateral panuveitis involving the posterior pole and peripheral fundus. Optical coherence tomography angiography (OCTA) provides several advantages over traditional fluorescence angiography for revealing pathological abnormalities of the retinal vasculature. Until recently, however, the OCTA field of view (FOV) was limited to 6 × 6 mm2 scans.Purpose: This study examined retinal vasculature and choriocapillaris abnormalities across multiple regions of the retina (15 × 9 mm2 wide field, macular, peripapillary regions) among acute and convalescent VKH patients using a novel widefield swept-source OCTA (WSS-OCTA) device and assessed correlations between imaging features and best-corrected visual acuity (BCVA).Methods: Twenty eyes of 13 VHK disease patients in the acute phase, 30 eyes of 17 patients in the convalescent phase, and 30 eyes of 15 healthy controls (HCs) were included in this study. Vascular length density (VLD) in superficial and deep vascular plexuses (SVP, DVP), vascular perfusion density (VPD) in SVP, DVP, and choriocapillaris (CC), and flow voids (FV) in CC were measured across multiple retinal regions via WSS-OCTA (PLEX Elite 9000, Carl Zeiss Meditec Inc., USA) using the 15 × 9 mm2 scan pattern centered on the fovea and quantified by ImageJ.Results: Compared to HCs, acute phase VKH patients exhibited significantly reduced SVP-VLD, SVP-VPD, and CC-VPD across multiple retinal regions (all p < 0.01). Notably, the FV area was more extensive in VKH patients, especially those in the acute phase (p < 0.01). These changes were reversed in the convalescent phase. Stepwise multiple linear regression analysis demonstrated that macular DVP-VLD and macular CC-VPD were the best predictive factors for BCVA in the acute and convalescent VKH groups.Conclusion: The wider field of SS-OCAT provides more comprehensive and detailed images of the microvasculature abnormalities characterizing VKH disease. The quantifiable and layer-specific information from OCTA allows for the identification of sensitive and specific imaging markers for prognosis and treatment guidance, highlighting WSS-OCTA as a promising modality for the clinical management of VKH disease.

Comparison of the clinical features between posterior scleritis with exudative retinal detachment and Vogt-Koyanagi-Harada disease.

This study aims to analyse the differences in clinical characteristics between VKH disease and PS with exudative retinal detachment (ERD). The medical records of 18 eyes of 12 patients with PS accompanied by ERD and 32 eyes of 16 patients with VKH disease were retrospectively reviewed. Single ERD was more common in PS, while hyperreflective dots, multiple ERD, retinal pigment epithelium folds were more common in VKH disease on OCT. Both posterior coat thickness and choroid thickness were higher in VKH eyes. "T" sign was observed in 6 of 18 eyes (33.3%) in the PS group, whereas in none of the eyes of VKH disease. No significant differences were shown in FA imaging between PS and VKH cases. Relapse occurred in 12 eyes (66.7%) in PS group, mainly in the posterior segment, while 6 eyes (18.8%) experienced recurrence in the anterior segment in VKH group. There are characteristic differences in multimodal imaging parameters and clinical course between VKH and PS with ERD.

Widefield Swept-Source Optical Coherence Tomography Angiography Assessment of Choroidal Changes in Vogt-Koyanagi-Harada Disease.

Purpose: To investigate choroidal changes in patients with Vogt-Koyanagi-Harada disease (VKH) using widefield swept-source optical coherence tomography angiography (SS-OCTA).Methods: In this cross-sectional study, 133 eyes of 69 patients with VKH (52 eyes of 28 active VKH patients and 81 eyes of 41 inactive VKH patients) and 104 eyes of 52 age and sex matched healthy volunteers were imaged using a widefield SS-OCTA instrument. On 12 mm × 12 mm OCTA scans, mean choroidal thickness (MCT), choroidal vascularity index (CVI), choriocapillaris (CC) flow area, and mean retinal thickness (MRT) were separately calculated in the fovea (diameter of 1 mm) and in concentric rings with different radii (1–3, 3–6, 6–9, and 9–12 mm).Results: Eyes with active VKH showed significant increases in MCT, CVI, and MRT, and decreased CC flow area in all central and peripheral regions (0–1, 1–3, 3–6, 6–9, and 9–12 mm) than in the healthy eyes (p ≤ 0.01) and inactive VKH eyes (p < 0.05). Inactive VKH eyes only showed marked decrease in CC flow area in all regions compared with controls (p < 0.05). Flow voids were observed in 51 of 52 (98.1%) active VKH eyes and 50 of 81 (61.7%) inactive VKH eyes on 12 mm × 12 mm OCTA. The MCT of all regions was significantly correlated with age, disease duration, and disease activity, whereas CVI was associated with age and disease activity. The CC flow void was related to visual acuity in all regions (p < 0.05).Conclusion: Widefield SS-OCTA enables a more comprehensive evaluation of chorioretinal changes in patients with VKH disease. Structural and vascular abnormalities are observed in both the central and peripheral choroid and are closely correlated with disease activity.

Rheumatologic manifestations in a cohort of patients with Vogt-Koyanagi-Harada disease.

Vogt-Koyanagi-Harada Disease (VKHD) is a systemic autoimmune disorder characterized by granulomatous panuveitis. Inflammatory rheumatic diseases (IRDs) are among the differential diagnosis of VKHD. However, current knowledge on the rheumatological aspects of VKHD is still limited. We aimed to investigate the prevalence of rheumatic conditions in VKHD patients. VKHD patients were included in the study and they were reviewed in terms of the presence of any rheumatological manifestations. There were 18 patients with a female preponderance (83.3%, female). Inflammatory type of peripheral joint pain (11%) and sicca symptoms (33%) were the most common rheumatological findings. The frequency of spondyloarthritis-related features such as inflammatory back pain and HLA-B27 rate was not increased. None of the patients had radiographic sacroiliitis. Anti-nuclear antibody was positive in high titres nearly in 30% of the patients and three patients had antibodies against extractable nuclear antigens. Nailfold capillaroscopy was abnormal in about one-third of the patients. Pathergy test was negative in all cohorts. While angiotensin-converting enzyme was elevated in nearly 20% of the patients, there were no abnormalities on chest X-rays. VKHD shares some features with IRDs. The common features were mostly suggestive of connective tissue disease rather than SpA or rheumatoid arthritis.

Increased serum level of interleukin-33 in Vogt-Koyanagi-Harada correlates with disease activity

ObjectivesTo measure the serum level of IL-33 in patients with Vogt-Koyanagi-Harada disease (VKH) and Behçet's uveitis (BU) in the Chinese Han population and investigate its associations with disease activity and clinical parameters. MethodsSerum was collected from 41 VKH patients (16 active and 25 inactive patients), 60 BU patients (24 active and 36 inactive patients), and 36 healthy controls. The serum level of IL-33 was measured using the enzyme-linked immunosorbent assay (ELISA) method. Demographic features, clinical manifestations, and intraocular inflammation activity scores (anterior chamber cells score, anterior chamber flare score, and vitreal haze score) were recorded. ResultsThe serum level of IL-33 significantly increased in all VKH patients, active VKH patients, and inactive VKH patients, as compared to healthy controls (p < 0.001, p < 0.001, and p = 0.002, respectively), and was higher in the active VKH than in the inactive VKH patients (p = 0.049). The serum level of IL-33 positively correlated with the anterior chamber cells score, vitreal haze score, and the annualized number of relapses in VKH patients (Rho = 0.359, p = 0.021; Rho = 0.344, p = 0.028; Rho = 0.537, p < 0.001, respectively). Serum IL-33 level was significantly associated with the annualized number of relapses in patients with BU (Rho = 0.361, p = 0.005). ConclusionSerum IL-33 level is significantly increased in VKH patients in the Chinese Han population. IL-33 level is in positive correlation with the activity and relapses of VKH. Increased IL-33 might contribute to the pathogenesis of VKH and serve as a potential biomarker for VKH disease.

SARS-CoV-2 among the Potential Viral Triggers for Vogt-Konayagi-Harada Disease: First Case Report and Literature Review

ABSTRACT Objective To describe a case of Vogt–Koyanagi–Harada (VKH) disease associated with the novel coronavirus disease 2019 (COVID-19). Materials and methods A retrospective review of patient records was carried out to obtain data for a case report. A literature review was conducted of cases of uveitis associated with COVID-19 reported to date. Case description A 32-year-old Hispanic female presented with bilateral granulomatous panuveitis consisting of VKH disease 2 weeks after a clinical course of COVID-19. Both eyes presented with anterior uveitis and edematous hyperemic papilla with flame-shaped peripapillary hemorrhages, macula-off serous retinal detachments with subretinal fluid, and vitritis. A complete systemic work-up was performed to exclude infectious or inflammatory etiologies. Ocular inflammatory signs and systemic symptoms improved with corticosteroid and immunomodulatory therapy. Conclusion SARS-CoV-2 can be a potential immunological trigger for VKH disease. Patients presenting with panuveitis and a history of a previous flu-like illness should raise clinical suspicion of the presence of SARS-CoV-2 in the context of the ongoing global pandemic.

Initial-onset acute uveitis associated with Vogt-Koyanagi-Harada disease presenting with unilateral exudative retinal detachment despite bilateral choroidal involvement.

To investigate the frequency of initial-onset acute uveitis associated with Vogt-Koyanagi-Harada (VKH) disease presenting with unilateral exudative retinal detachment. A retrospective case series. During the period between January 1998 and December 2020, we identified 135 patients with initial-onset acute uveitis associated with VKH disease. Among them, 5 (3.7%) patients were referred to have unilateral uveitis due to the presence of exudative retinal detachment in only one eye. Optical coherence tomography confirmed the presence of unilateral exudative retinal detachment, however, indocyanine green angiography (ICGA) revealed characteristic findings of bilateral granulomatous choroidal inflammation typical for initial-onset acute uveitis associated with VKH disease. Patients with initial-onset acute uveitis associated with VKH disease can present with unilateral exudative retinal detachment. ICGA assessment of the choroid revealed the presence of subclinical involvement of the fellow eyes.

This issue of ACTA

This issue of ACTA

Long-term Outcomes of Uveitis Associated with Vogt-Koyanagi-Harada Disease in the Pediatric Age Group

ABSTRACT Purpose To investigate the outcomes of uveitis associated with Vogt-Koyanagi-Harada (VKH) disease in pediatric age group (aged 16 years and under). Methods A retrospective review of patients with VKH disease. Results Among the 244 patients identified, 38 (76 eyes) were children. Among them, five had insulin-dependent diabetes mellitus. 21 presented with initial-onset acute disease and 17 with chronic recurrent disease. The mean follow-up period was 59.1 months. At presentation, chronic recurrent disease was associated with more severe inflammation as indicated by the presence of mutton-fat keratic precipitates (p < .001), iris nodules (p = .005) and posterior synechiae (p < .001). During follow-up, the rate of complications was higher in children with chronic recurrent disease compared with initial-onset acute disease (p < .001). 92.4% of the eyes with initial-onset acute disease achieved a final visual acuity of ≥20/40 compared with 70.6% of the eyes with chronic recurrent disease (p = .013). Conclusions Chronic recurrent VKH disease in children is associated with worse outcomes.

Elevated Serum Immunoglobulin E Levels are Associated with the Severity of Newly Diagnosed, Acute Vogt–Koyanagi–Harada Disease

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

Vogt-Koyanagi-Harada disease in Spain.

To describe the clinical and epidemiological characteristics of patients with Vogt-Koyanagi-Harada (VKH) disease in Spain. This was a retrospective multicenter analysis of data from VKH patients followed for at least 6 months. The data collected were related to demographics, clinical manifestations, treatments, and complications. Participants were 112 patients (224 eyes), from 13 tertiary referral centers, of mean age 37.5 ± 14.7 years; 83.9% were women. Ethnicities were 61.6% Caucasian and 30.4% Hispanic. The disease was classified as complete in 16.1%, incomplete in 55.4%, and probable in 28.6%. When seen for the first time, the clinical course was acute in 69.6%, recurrent chronic in 15.2%, and chronic in 14.3%. The most frequent treatment was corticosteroids (acute stage 42.2%, maintenance stage 55.6%). The most common complications were cataract (41.1%) and ocular hypertension (16.1%). In most eyes, visual acuity was improved (96.7%) or remained stable at the end of follow up. VKH in Spain mostly affects women and presents as incomplete acute stage disease. Visual prognosis is good. Cataract and glaucoma are the two most frequent complications.

Vogt Koyanagi Harada Disease In Paediatric Age Group: Clinical Characteristics, Remission, Recurrences and Complications in Asian Indian Population

ABSTRACT Purpose To describe disease characteristics and outcomes of Vogt-Koyanagi-Harada (VKH) syndrome in paediatric patients. Study Design Retrospective chart analysis Methods: A retrospective Analysis of all patients ≤16 years with VKH syndrome was done. Clinical presentations, complications, recurrences and outcomes in cases of paediatric VKH were reviewed. Results 72 eyes of 36 patients with a mean age at presentation of 13.7 ± 2.34 years were assessed. Mean duration of symptoms and follow up were 9.88 ± 17.3 weeks and 55 months respectively. Clinical signs at presentation included anterior chamber cells >2+(34/72eyes, 47.2%), granulomatous keratic precipitates (6 eyes, 8.3%), posterior synechiae (35 eyes,48.6%), disc edema (46 eyes, 63.8%), neurosensory retinal detachments (44 eyes, 61.1%) and ‘sunset-glow’ fundus (9 eyes, 12.5%). Best corrected visual acuity (BCVA) at the time of presentation was 1.3logMAR or a Snellens equivalent of 20/400 which improved to 0.51logMAR (Snellens equivalent of 20/63) at last follow up. Remission was achieved in 61.1% cases. More than half of our patients developed one or more complications. Conclusion VKH in paediatric patients poses a challenge due to a delayed presentation and paediatric VKH patients have a worse visual acuity at the time of presentation as compared to adult age groups. Rates of remission may be low along with high risk of complications and hence there is a need for prolonged immunosuppression.

Decrease of choriocapillary vascular density measured by optical coherence tomography angiography in Vogt-Koyanagi-Harada disease

PurposeChanges of choroidal circulation throughout the disease course of Vogt-Koyanagi-Harada (VKH) disease and the clinical significance remain unclear. Choriocapillary vascular density (CC VD) measured by optical coherence tomography angiography (OCTA) were compared in different disease stages of VKH and its correlation with other parameters was analyzed, aiming to explore their clinical relevance.MethodsThis is a retrospective case series. One hundred and fourteen VKH patients and 47 normal controls (NCs) were included. Patients were grouped into the acute uveitic, convalescent, and chronic recurrent stages (only anterior recurrent cases included), and OCTA images were obtained from VKH patients in these stages. Best corrected visual acuity (BCVA), CC VD, and subfoveal choroidal thickness (SFCT) were recorded and compared.ResultsCC VD in acute (58.26% ± 0.84%), convalescent (64.85% ± 0.33%), and chronic recurrent (62.78% ± 0.70%) stage of VKH patients were all significantly lower than that in NCs (66.37% ± 0.41%) (p < 0.001, p = 0.017, and p < 0.001, respectively). CC VD increased by 6.59% ± 0.91% with resolution of acute inflammation (p < 0.001) and decreased by 2.07% ± 0.74% during anterior uveitis relapse (p = 0.009). Patients with a positive history of anterior recurrence had lower CC VD (− 2.43% ± 0.75%, p = 0.003) in the convalescent stage than those without. CC VD was negatively correlated with logMAR BCVA in VKH (r = − 0.261, p < 0.001).ConclusionCC VD was decreased in every stage of VKH. CC VD has the potential to reflect the status of uveitis and might be promising in monitoring the disease activity. OCTA is a convenient and straightforward tool to evaluate choroidal vascularity, and CC VD provides supplemental quantitative information of the choriocapillaris. Further studies are needed to explore the values of OCTA quantitative parameters in monitoring VKH progression, predicting visual prognosis, and guiding clinical decisions.