Vogt-Koyanagi-Harada Research Articles

Pattern of non-infectious uveitis and visual outcome in a tertiary care university-based hospital: A multidisciplinary approach

Aim of the workTo study the pattern of non-infectious uveitis (NIU) in different systemic autoimmune diseases and to determine the visual outcome and complications in relation to systemic immunomodulatory therapy. Patients and methodsThe study was conducted on 105 NIU patients with different autoimmune diseases: Behçet’s disease (BD), Vogt–Koyanagi–Harada (VKH), juvenile idiopathic arthritis (JIA), sarcoidosis, idiopathic uveitis and ankylosing spondylitis (AS). All patients were subjected to full ophthalmological examination. ResultsThe frequency of NIU was highest in BD (n = 61;58.1 %) followed by VKH (n = 24;22.9 %), JIA (n = 8;7.6 %), sarcoidosis (n = 7;6.7 %), idiopathic uveitis (n = 4;3.8 %) and AS(n = 1;1%) and their mean age at NIU onset was 30 ± 12 years. Panuveitis (PanU) was identified in 84 (80 %), anterior uveitis (AU) in 11(10.5 %), posterior uveitis (PU) in 9 (8.6 %) and intermediate uveitis (IU) in one (1 %) patient. A chronic recurrent course was detected in 98 (93.3 %) patients and bilateral involvement was present in 86 (81.9 %). Laterality of eye affection (unilateral or bilateral) was not related to eye activity or type of uveitis. The most common complication was cataract (17.1 %). A significant relation was found between the control of eye activity and immunosuppression intake in the last 6 months (p = 0.002). 38 (36.2 %) patients were considered legally blind. A non-significant relation was found between legal blindness with type of uveitis (p = 0.82), immunosuppressives(p = 0.06) or biological therapy (p = 0.35). ConclusionThe most common pattern of NIU in systemic autoimmune diseases was panuveitis. Behçet’s disease and VKH disease were the most frequent etiologies for NIU and cataract was the most common complication.

Pertussis toxin-induced inhibition of Wnt/β-catenin signaling in dendritic cells promotes an autoimmune response in experimental autoimmune uveitis

BackgroundPrevious reports have indicated that disrupting the Wnt/β-catenin pathway in dendritic cells (DCs) may affect the progression of autoimmune inflammation; however, the factors and timing that regulate Wnt/β-catenin signaling have not been clearly understood.MethodsExperimental autoimmune uveitis (EAU) mice and Vogt–Koyanagi–Harada disease (VKH) patient samples were used to detect the expression of Wnt/β-catenin pathway genes. Western blot, real-time PCR, flow cytometry, and ELISA were performed to examine the expression of components of the Wnt/β-catenin pathway and inflammatory factors. DC-specific β-catenin knockout mice and 6-bromoindirubin-3′-oxime (BIO) administered mice were used to observe the effect of disrupting the Wnt pathway on EAU pathogenesis.ResultsWnt/β-catenin signaling was inhibited in DCs during the induction phase of EAU. The inhibition was mediated by pertussis toxin (PTX), which promoted DC maturation, in turn promoting pathogenic T cell proliferation and differentiation. In vivo experiments confirmed that deleting β-catenin in DCs enhanced EAU severity, and pre-injection of PTX advanced EAU onset. Administration of a Wnt activator (BIO) limited the effects of PTX, in turn ameliorating EAU.ConclusionsOur results demonstrate that PTX plays a key role as a virulence factor in initiating autoimmune inflammation via DCs by inhibiting Wnt/β-catenin signaling in EAU, and highlight the potential mechanism by which infection can trigger apparent autoimmunity.

An audiological profile in vogt-koyanagi-harada syndrome

The Vogt-Koyanagi-Harada syndrome (VKHS) is a rare, multisystemic, granulomatous inflammatory and autoimmune disease that affects the pigmented structures such as eyes, ears, skin, and hair. The syndrome mainly affects the eyes, followed by bilateral chronic panuveitis. A 34 years old female came with complaints of reduced vision and reduced hearing sensitivity to our hospital. She was diagnosed with VKHS in the ophthalmology department. Throughout the audiologist assessment, she presented bilateral sensorineural hearing loss, absent otoacoustic evoked emissions, and complaints about postural vertigo and acute tinnitus. The specific case reported presented sudden sensorineural hearing loss, vertigo, tinnitus, and bilateral ocular disease. Even though drug treatment was performed, the hearing loss remained.

Quality of Life (QoL) in Non-Acute Vogt–Koyanagi–Harada Disease (VKHD) at Two Time Points 24 Months Apart

ABSTRACT Aims To investigate the changes in quality-of-life (QoL) metrics at a 24-month interval in non-acute VKHD patients and their association with inflammation, treatment, and visual function. Methods SF-36 and VFQ-25 questionnaires were administered at two 24-month-apart moments to 22 non-acute VKHD patients followed for ≥12 months since acute disease onset. “Improvement,” “unchanged,” or “worsening” in questionnaires scores (difference >5-point) between M1 and M2 and their associations were sought. Results Absence of systemic treatment or optic disc hyperfluorescence was associated with improved general health (SF-36). Improvement in binocular contrast sensitivity resulted in better ocular pain score; absence of anterior uveitis relapse, stable fundus findings, no use of cyclosporine or no intravitreal injections resulted in unchanged/better dependency score; no intravitreal injections resulted in unchanged/better mental health score (VFQ-25). Conclusion Stability/improvement in QoL scores was associated with controlled inflammation, better visual function, and no need for treatment. Subclinical inflammatory signs did not impact QoL scores.

Predictive Factors for the Prognosis of Vogt-Koyanagi-Harada Disease

ABSTRACT Purpose To identify the prognostic factors in Vogt–Koyanagi–Harada (VKH) disease. Methods This study included 23 patients (46 eyes) with acute-phase VKHdivided into two subgroups: Group 1; acute-resolved (10 patients), group 2; chronic-recurrent (13 patients). Results Mean age were 29.5 ± 10.2 years in group 1, 35.8 ± 12.2 years group 2 (p = .033). Best-corrected visual acuity with logMAR at admission was 0.91 ± 0.65 in group 1, 0.88 ± 0.62 in group 2 (p = .798), and improved to 0.08 ± 0.24, 0.18 ± 0.6, respectively (p = .557). Extraocular findings were detected in 30% in group 1, and 53.8% in group 2 at the time of admission (p = .108). All patients were treated with 1 g/day 3–5 days intravenous steroid, and 10 patients had immunomodulatory treatment. Initiation of immunomodulatory therapy did not affect the prognosis (p = .676). Conclusion Older patients and/or who developed extraocular findings at the presentation were more prone to show recurrences.

Autoimmune uveitis in Behçet's disease and Vogt-Koyanagi-Harada disease differ in tissue immune infiltration and T cell clonality.

Non-infectious uveitis is often secondary to systemic autoimmune diseases, with Behçet's disease (BD) and Vogt-Koyanagi-Harada disease (VKHD) as the two most common causes. Uveitis in BD and VKHD can show similar clinical manifestations, but the underlying immunopathogenesis remains unclear. To understand immune landscapes in inflammatory eye tissues, we performed single-cell RNA paired with T cell receptor (TCR) sequencing of immune cell infiltrates in aqueous humour from six patients with BD (N = 3) and VKHD (N = 3) uveitis patients. Although T cells strongly infiltrated in both types of autoimmune uveitis, myeloid cells only significantly presented in BD uveitis but not in VKHD uveitis. Conversely, VKHD uveitisbut not BD uveitis showed an overwhelming dominance by CD4+ Tcells (> 80%) within the T cell population due to expansion of CD4+ T cell clusters with effector memory (Tem) phenotypes. Correspondingly, VKHD uveitis demonstrated a selective expansion of CD4+ T cell clones which were enriched in pro-inflammatory Granzyme H+ CD4+ Tem cluster and showed TCR and Th1 pathway activation. In contrast, BD uveitis showed a preferential expansion of CD8+ T cell clones in pro-inflammatory Granzyme H+ CD8+ Tem cluster, and pathway activation for cytoskeleton remodelling, cellular adhesion and cytotoxicity. Single-cell analyses of ocular tissues reveal distinct landscapes of immune cell infiltration and T-cell clonal expansions between VKHD and BD uveitis. Preferential involvements of pro-inflammatory CD4+ Th1 cells in VKHD and cytotoxic CD8+ T cells in BD suggest a difference in disease immunopathogenesis and can guide precision disease management.

Common practice patterns in the diagnosis and management of Vogt-Koyanagi-Harada syndrome: a survey study of uveitis specialists.

Vogt-Koyanagi-Harada (VKH) syndrome is an inflammatory condition characterized by bilateral, granulomatous panuveitis with or without systemic manifestations, and accounts for up to 18% of referrals for panuveitis at tertiary centers in the United States of America. Despite ongoing research, there is limited evidence and no clear consensus on how to diagnose and treat patients with VKH, leading to variations in practice patterns among uveitis specialists. An anonymous, online survey was distributed to uveitis specialists in the American Uveitis Society (AUS). The survey included 21 questions that asked for non-identifiable demographics and covered topics such as preferred imaging modalities, treatment for the first episode of VKH, and perceived efficacy of immunomodulatory therapy (IMT). A total of 104 surveys were included for analysis, representing a 38.4% response rate from the AUS listserv. A majority of respondents were uveitis fellowship trained and practiced in North America in an academic setting. Fluorescein angiography and enhanced depth imaging with optical coherence tomography were rated as the most consistently useful methods for the diagnosis of VKH. For treatment of acute initial-onset VKH, responses were divided between a preference for high-dose systemic corticosteroids with IMT (61.5%) and without IMT (37.5%). Methotrexate and mycophenolate mofetil were the most common IMTs to be used as first-line therapies, but adalimumab and infliximab were perceived to be the most effective for the treatment for VKH. While there are some common trends in the practice patterns for the diagnosis and treatment of patients with VKH, there was no clear consensus on the topic of IMT. There was a slight preference among uveitis specialists to use both IMT and systemic corticosteroids for the first episode of acute VKH.

Clinical Profile of Patients with Vogt–Koyanagi-Harada Disease at a Tertiary Eye Hospital in Bangladesh

Background: Vogt-Koyanagi-Harada (VKH) disease is an autoimmune inflammatory disease characterized by bilateral panuveities affecting the young adults. Approximately 3% of the uveitis patients are VKH patients. Objective: To assess the clinical profile of patients with Vogt-Koyanagi- Harada (VKH) disease.

Prospective Study of Clinical Characteristics and Visual Outcome in Vogt–Koyanagi–Harada Syndrome in a Tertiary Eye Hospital in South India

Purpose: To describe the demographic pattern, clinical characteristics, treatment modalities, and visual outcome in Vogt–Koyanagi–Harada (VKH) disease patients observed at a tertiary eye care centre in South India. Methods: A prospective chart analysis of 38 patients with VKH disease. Results: Of the 38 VKH patients, 31 (81.6%) were female, the mean age was 39.8 years, and the median duration of symptoms was 10 days (range: 2 to 180 days). The most common ocular complaint was a defective vision (70 eyes or 92.1%). Extraocular manifestations were headache (13 patients or 34.2%) and tinnitus (4 patients or 10.5%). Twenty-four patients (63.2%) presented with bilateral panuveitis, and 12 patients (31.6%) had posterior uveitis. The most common posterior segment presentation included bilateral serous retinal detachment (100%), disc oedema (37 eyes or 48.7%), and vitritis (31 eyes or 40.8%). All patients (100%) were treated with oral and topical prednisolone, based on the ocular inflammation status, 9 patients (23.7%) required dexamethasone (intramuscular), 39 eyes (51.3%) required posterior subtenon triamcinolone, 16 patients (28.9%) required methotrexate, 1 (2.6%) required cyclophosphamide, 1 (2.6%) required azathioprine. At the final visit, the majority of the patients had statistically significant good visual outcomes. Conclusion: Prompt diagnosis and early initiation of treatment with systemic, local steroid and immunosuppressive therapy showed faster recovery with good visual outcome.

Clinical and multimodal imaging characteristics of eyes with Vogt-Koyanagi-Harada disease: An Egyptian experience.

Vogt-Koyanagi-Harada (VKH) disease is a vision-threatening inflammatory disorder that is challenging in diagnosis and management. Retrospective, record-based analysis of 54 eyes belonging to 27 adult patients that fulfilled the revised diagnostic criteria for VKH between January 2018 and January 2021. Demographic, clinical, and imaging data on presentation and during follow-up visits were collected for each patient. Available imaging studies included B-scan ultrasonography (B-scan US), spectral domain optical coherence tomography (OCT), fundus fluorescein angiography (FFA), and OCT angiography (OCT-A). The female-to-male ratio was 2.38:1. Nineteen patients (70.37%) presented during an initial attack, while eight patients (29.63%) presented during recurrence. The most commonly presenting sign in the posterior segment was exudative retinal detachment (44 eyes, 81.48%). B-scan US was utilized in 4 eyes (7.41%), OCT was utilized in 48 eyes (88.89%) with the most common finding being subretinal fluid (43 eyes, 89.58%), FFA was performed in 39 eyes (72.22%) with the most common finding being punctate hyperfluorescence and late dye pooling (33 eyes, 84.62%), and OCT-A was performed in 30 eyes (55.56%), in which choriocapillaris flow deficit that correlated with disease activity was detectable in 25 eyes (83.33%). Improved visual acuity was noted in 85% of the eyes that were followed up. Early diagnosis and treatment of VKH result in favorable visual outcome. Multimodal imaging, with the recent addition of OCT-A, provides complementary data that could serve in diagnosis and monitoring.

Panophthalmitis After Aurolab Aqueous Drainage Implant (AADI) in Refractory Glaucoma with Vogt-Koyanagi-Harada Syndrome (VKH): A Case Report

This case of Vogt-Koyanagi-Harada (VKH) disease underwent Aurolab Aqueous Drainage Implant (AADI) for refractory glaucoma. Later, developed postoperative (2 months) panophthalmitis characterized by sudden visual loss, moderate pain, redness, and purulent discharge. Systemic, topical, and intravitreal antibiotic therapy was started immediately, and evisceration was performed.

Changes of Peripapillary Capillary Density in Patients with Vogt–Koyanagi–Harada Disease Evaluated by Optical Coherence Tomography Angiography

To analyze the longitudinal changes in peripapillary capillary density in patients with acute VKH with or without optic disc swelling by optical coherence tomography angiography (OCTA). Retrospective case series. 44 patients (88 eyes) were enrolled and were divided into two groups according to presence/absence of optic disc swelling before treatment. Peripapillary capillary images were obtained by OCTA before and after 6 months of corticosteroid treatment and used to determine the radial peripapillary capillary (RPC), retinal plexus, and choriocapillaris vessel perfusion densities. Optic disc swelling was present in 12 patients (24 eyes) and absent in 32 patients (64 eyes). The sex distribution, age, intraocular pressure, and best-corrected visual acuity before and after treatment were not significantly different between the two groups (all P > 0.05). Compared to those in nonoptic disc swelling group, the percentages of decreased vessel perfusion densities after treatment in the supranasal (RPC, 100.00% vs. 75.00%), infranasal (RPC, 100.00% vs. 56.25%), infratemporal (RPC, 66.67% vs. 37.50%), and infranasal quadrants (retinal plexus, 83.33% vs. 56.25%) were significantly more in optic disc swelling group. The choriocapillaris vessel perfusion density increased after treatment in both groups. Decreases in vessel perfusion densities of the RPC and retinal plexus after treatment in VKH patients with optic disc swelling were more common than in those without optic disc swelling. The choriocapillaris vessel perfusion density increased after treatment, regardless of the presence/absence of optic disc swelling.

Clinical Variations of Uveitis in Immuno-Inflammatory Diseases. Review of the Literature. Part 2

In the previous part of the review clinical and diagnostic aspects of some non-infectious uveitis in patients with immunoinflammatory diseases were discussed. In this part we proceed the discussion of ocular manifestations of a number of other immunoinflammatory conditions. In addition to uveitis associated with spondyloarthropathies, rheumatoid arthritis, Still’s disease, juvenile idiopathic arthritis and systemic sarcoidosis described in the previous part, ocular manifestations are also common in systemic vasculitis, systemic lupus erythematosus, Vogt—Koyanagi—Harada syndrome. Despite the numerous diagnostic schemes and therapy algorithms developed to date, much in the pathogenesis of uveitis associated with immuno-inflammatory diseases remains unclear. The need to develop personalized and multidisciplinary approaches for the treatment and diagnosis of non-infectious uveitis in numerous systemic immunoinflammatory diseases remains relevant. In-depth understanding of etiopathogenetic mechanisms of immunoinflammatory processes will allow to develop new approaches in the treatment of patients with uveitis.

IPSC-based model of Vogt-Koyanagi-Harada disease for phenotype recapitulation and drug screening

Vogt-Koyanagi-Harada (VKH) disease, a major blinding eye disease, is characterized by an autoimmune response against melanocytes in multiple organs throughout the body. Currently, the aetiology and pathogenesis of VKH disease are unclear, and the treatment strategy needs to be further optimized. The retinal pigment epithelium (RPE), a monolayer of pigmented cells of the fundus, is essential for maintaining normal visual function and is involved in both the acute and chronic stages of VKH disease. Therefore, the functions of the RPE may play a critical role in the aetiology and treatment of VKH disease. Herein, we established a human induced pluripotent stem cell (hiPSC) RPE model of VKH disease by reprogramming peripheral blood mononuclear cells (PBMCs) into iPSCs and then differentiating them into RPE cells. Patient-derived RPE cells exhibited barrier disruption, impaired phagocytosis, and depigmentation compared with those from normal controls, which was consistent with the features of VKH disease. Furthermore, a small molecular compound targeting EGR2 was found to rescue the barrier and phagocytic functions of the hiPSC-RPE cells through high-throughput virtual screening and functional studies, suggesting a promising strategy for the treatment of VKH disease.

Peripheral immune landscape and natural killer-like B cells in human Vogt-Koyanagi-Harada disease

AbstractVogt-Koyanagi-Harada (VKH) disease is a systemic autoimmune disorder threatening the eyesight. The pathogenic mechanisms and biomarkers reflecting disease severity and predicting treatment response require further exploration. Here, we performed a single-cell analysis of peripheral blood mononuclear cells (PBMC) obtained from eight patients with VKH disease and eight healthy controls to comprehensively delineate the changes in VKH disease. We showed a mixture of inflammation, effector, and exhausted states for PBMCs in VKH disease. Notably, our study implicated a newly identified B cell subset, natural killer-like B cells (K-BC) characterized by expressing CD19 and CD56, was correlated with VKH disease. K-BCs expanded in VKH disease, fell back after effective treatment, and promoted the differentiation of pathogenic T cells. Overall, we mapped the peripheral immune cell atlas in VKH disease and indicated the pathogenic role and potential value in predicting treatment response of K-BCs.

Healing features of outer retinal layers on SS OCT in VKH disease

AbstractPurpose: To describe restoration of outer retinal layers on Swept‐Source OCT (SS OCT) in Vogt Koyanagi Harada (VKH) disease over a year after the disease onset.Methods: Prospective longitudinal study carried out at the department of Ophthalmology of Fattouma Bourguiba University Hospital of Monastir. Twenty‐one patients (42 eyes) were followed‐up for 12 months after the disease onset. The patients were included at the acute phase and subsequently treated. Sequential SS OCT changes were assessed.Results: Structural SS OCT changes occurred in 24 eyes (57.1%) on month 3 (M3), in 23 eyes (54.8%) on month 6 (M6), and in 19 eyes (45.2%) on month 12 (M12). They included external nuclear layer atrophy (14.3% on M3; 11.9% on M6; and 9.5% on M12); ellipsoid zone disruption (40.5% on M3; 38.1% on M6; and 33.3% on M12); interdigitation zone disruption (42.9% on M3; 40.5% on M6; and 33.3% on M12); and multifocal retinal pigment epithelium thickening (50% on M3; 45.2% on M6; and 31% on M12). No significant association between final visual acuity and external retinal layers alterations at any stage of the study was found. Stepwise analysis showed that the presence of serous retinal detachment at presentation was an independent factor for structural SS OCT changes on M3.Conclusions: After initial onset, SS OCT shows thinning of the outer nuclear layer, disruption of the ellipsoid and interdigitation zones, and multifocal retinal pigment epithelium thickening. Serous retinal detachment at presentation is an independent factor for structural SS OCT changes during the first year of the disease.

EFFICACY OF COMBINED SYSTEMIC CORTICOSTEROID AND EARLY IMMUNOMODULATORY THERAPY WITHIN THREE MONTHS OF ONSET IN VOGT-KOYANAGI-HARADA DISEASE.

To evaluate the clinical outcomes of combined systemic corticosteroid and early immunomodulatory therapy (IMT) within 3 months of onset in Vogt-Koyanagi-Harada (VKH) disease compared with conventional therapy. This retrospective observational case series included 73 eyes of 38 patients with VKH, categorized into the conventional (n = 41) and the early IMT (n = 32) groups. Clinical information was gathered from patients' medical records. Primary outcome measures were visual acuity, subfoveal choroidal thickness, and uveitis outcome including occurrence of sunset glow fundus. The logarithm of minimal angle of resolution visual acuity of both groups improved, with statistically significant difference at the last follow-up (P < 0.01, Mann-Whitney U test). Their mean subfoveal choroidal thickness decreased, with no statistically significant difference at the last follow-up (P = 0.21, T-test). In the conventional and early IMT groups, 27 (65.9%) and 15 (46.9%) eyes, respectively, had chronic or chronic recurrent VKH and sunset glow fundus was observed in 33 (80.5%) and 16 (50.0%) eyes, respectively. Combined systemic corticosteroid and early IMT within 3 months of onset was superior to conventional therapy in the final visual and uveitis outcome of patients with VKH disease. Therefore, IMT may be added early in cases of VKH disease, even when under control with high-dose corticosteroid.

Vogt‐Koyanagi‐Harada disease following a serology‐proven Bartonella henselae retinitis: A case report

AbstractPurpose: To report a case of Vogt‐Koyanagi‐Harada (VKH) disease following a serology‐confirmed bartonella henselae retinitis.Methods: A single case report.Results: A 41‐year‐old female patient presented with a central scotoma in her right eye (RE). Fundoscopic examination showed a focal retinitis with a macular star in the RE. Work‐up revealed positive bartonellosis serology. The patient was treated with oral azithromycine and oral steroids during 4 weeks with subsequent healing of the retinitis lesion. Three months after initial presentation, the patient complained of loss of vision in both eyes. Ophthalmological examination showed a best‐corrected visual acuity (BCVA) of 20/200 on both eyes, with a bilateral granulomatous panuveitis with bilateral multifocal exudative retinal detachment. Results of multimodal imaging were consistent with the diagnosis of acute VKH disease.Given the history of positive bartonellosis serology, the patient was treated with doxycycline (200 mg/day) for 6 weeks in association with intravenous methylprednisolone for 5 days followed by oral steroids and progressive tapering. At three‐month follow‐up, BCVA improved to 20/20 in both eyes associated with resolution of clinical and structural OCT changes.Conclusions: Our case suggests that bartonella henselae infection might be a non‐previously reported infectious trigger of VKH disease in genetically predisposed patients. Alternatively, bartonella henselae infection might manifest with panuveitis mimicking VKH disease.

Bacillary Layer Detachment in Acute Vogt-Koyanagi-Harada Disease.

To evaluate the frequency and treatment response of eyes with bacillary layer detachment (BLD) in acute Vogt-Koyanagi-Harada (VKH) disease using spectral-domain optical coherence tomography (SD-OCT). We retrospectively reviewed the medical records of 58 eyes of acute VKH patients with at least 6 months of follow-up between January 2009 and March 2021. SD-OCT, color fundus photographs, and fluorescein angiography images were analyzed in all patients. The study included 58 eyes of 29 patients. BLD was detected in 33 of the 58 eyes (56.9%) at baseline. Mean serous retinal detachment (SRD) height was 918.50±336.64 μm in the BLD group and 215.33±167.83 μm in the group without BLD (p<0.05). A positive correlation was found between SRD height and the presence of BLD (r=0.783, p<0.05). BLD was significantly more common in patients with a baseline SRD height greater than 500 μm (p<0.05). As subfoveal central choroidal thickness (CCT) could not be measured by enhanced depth imaging-OCT at baseline due to extreme choroidal thickness in all eyes, the earliest post-treatment CCT measurements were analyzed. At the completion of pulse steroid therapy, mean CCT was 425±82.87 μm in the BLD group and 385.58±82.87 μm in the group without BLD (p=0.04). The mean time to BLD resolution was 12.88±6.5 days (range: 2-26). BLD is a common tomographic finding in eyes with acute VKH disease and can be differentiated from the associated SRD through careful SD-OCT analysis. Though it is mostly observed in patients with more serious disease, the presence of BLD has no negative effect on long-term visual function.

Comparison of the Three Sets of Diagnostic Criteria for Vogt–Koyanagi–Harada Disease in Southeast China – A Retrospective Case–Control Study

ABSTRACT Purpose To compare the utility of the Chinese diagnostic criteria for Vogt–Koyanagi–Harada (VKH) disease (CDCV), the revised diagnostic criteria (RDC) and the classification criteria by SUN (SUN-C). Methods Two groups of patients (VKH group and non-VKH group) were assessed in this retrospective case–control study. Sensitivity, specificity and area under receiver operating characteristic curve (AUC) were evaluated among these criteria. Results 258 patients were included after propensity score matching. The sensitivities were 92.2% in CDCV, 66.7% in RDC, and 54.3% in SUN-C. In different disease stages (early and late), similar sensitivity results were observed. The specificities were 96.1% in CDCV, 97.7% in RDC, and 99.2% in SUN-C. The AUCs were 0.942 in CDCV, 0.822 in RDC and 0.767 in SUN-C. Conclusion A higher sensitivity value and larger AUC in CDCV were found. CDCV are highly useful in the diagnosis and classification of VKH disease in Chinese patients.