Polyautoimmunity manifest as inflammatory myopathy, uveitis, and progressive cutaneous depigmentation in a mixed breed dog: a case report.

BACKGROUND: Polyautoimmunity is the expression of more than one autoimmune disease in a single patient. This report documents polyautoimmunity in a mixed breed dog with concurrent uveitis, cutaneous depigmentation, and inflammatory myopathy. CASE PRESENTATION: A 1-year-old male neutered mixed breed dog was presented for progressive generalized leukotrichia and leukoderma, bilateral panuveitis, and masticatory muscle atrophy. The latter progressed to myositis of lingual, pharyngeal, and masticatory muscles confirmed by biopsy. Temporalis muscle was completely replaced by adipose and fibrous tissue, and necrotic myofibers with extensive infiltration of mononuclear cells indicated active myositis of lingual muscle. Skin biopsies showed severe melanin clumping in epidermis, hair follicles, and hair shafts, and perifollicular pigmentary incontinence. Uveitis, depigmentation, and myositis affecting the masticatory, pharyngeal, and tongue muscles were diagnosed based on clinical, histological, and laboratory findings. CONCLUSIONS: To the authors' knowledge, this is the first report of concurrent uveitis, progressive cutaneous depigmentation, and inflammatory myopathy in a dog.

Identification of Hif1α as a Potential Participant in Autoimmune Uveitis Pathogenesis Using Single-Cell Transcriptome Analysis.

Purpose: This study purposed to depict the transcriptional changes associated with autoimmune uveitis (AU) pathogenesis and identify potential therapeutic targets of this disease. Methods: An experimental AU (EAU) model was established with retina antigen and adjuvants. An EAU control group was established with adjuvant only to eliminate nonspecific effects. We conducted single-cell RNA sequencing (scRNA-seq) on cervical draining lymph node cells of EAU, EAU control, and normal mice to identify the EAU-associated transcriptional changes and the potential pathogenic molecules. Subsequent flow cytometry, adoptive transfer experiment, scRNA-seq analysis of human uveitis, and proliferation assessment were conducted to verify the function of the interested molecule in uveitis. Results: The scRNA-seq data suggested that hypoxia-inducible factor 1 alpha (Hif1α) may participate in EAU pathogenesis via regulating T helper (Th)-17, Th1, and regulatory T cells. Hif1α inhibition alleviated EAU symptoms and regulated Th17, Th1, and regulatory T cell proportions. CD4+ T cells with repressed Hif1α expression failed to transfer EAU to naïve mice. In Vogt-Koyanagi-Harada disease, which is a human uveitis, Hif1α was also increased in CD4+ T cells and regulated their proliferation. Conclusions: The results indicate that Hif1α may participate in AU pathogenesis and are, thus, a potential therapeutic target.

Different MicroRNA profiles in Peripheral Blood mononuclear cells from patients with initial-onset and recurrent vogt-Koyanagi-Harada Disease.

BACKGROUND: Vogt-Koyanagi-Harada (VKH) disease is a common type of uveitis that leads to blindness. The clinical manifestations and treatment solutions are different between initial-onset and recurrent VKH. Therefore, identifying the microRNA (miRNA) profiles from initial-onset and recurrent VKH patients may shed light on the molecular mechanisms underlying the pathogenesis of VKH disease. METHODS AND RESULTS: RNAs isolated from peripheral blood mononuclear cells (PBMCs) from patients with initial-onset VKH, recurrent VKH, and healthy individuals were subjected to high-throughput miRNA sequencing. Pairwise analysis of miRNA sequencing data between groups was conducted to identify differentially expressed miRNAs (DEMs), which were verified using real-time quantitative polymerase chain reaction. After receiver operating characteristic analyses, we found that hsa-miR-4664-3p, hsa-miR-7704, hsa-miR-4504, and hsa-miR-206 may serve as biomarkers of different VKH stages. DEMs were classified into three groups based on their differential expression: DEMs in initial-onset stage, DEMs in recurrent stage, and DEMs common between both VKH stages (shared DEMs). Pathway enrichment analysis using the Kyoto Encyclopedia of Genes and Genomes identified the mitogen-activated protein kinase, tumor necrosis factor, and mechanistic target of rapamycin kinase pathways as significantly enriched among the target genes of recurrent stage and shared DEMs. Furthermore, we mapped a network of competing endogenous RNAs for hsa-miR-206, which we used to identify putative targets for VKH treatment. CONCLUSION: Hsa-miR-4664-3p, hsa-miR-7704, hsa-miR-4504, and hsa-miR-206 may serve as biomarkers for different stages of VKH. Additionally, our competing endogenous RNA network of hsa-miR-206 provides a new direction for VKH treatment.

The emerging role of epigenetics and gut microbiota in Vogt-Koyanagi-Harada syndrome.

Vogt-Koyanagi-Harada (VKH) syndrome is an autoimmune disorder characterized often by acute diffuse uveitis, also known as idiopathic uveoencephalitis. The associated complications can potentially affect multiple systems throughout the body, including eyes, ears, skin and nervous system. Although the pathogenesis of VKH syndrome remains unclear, it has been established that the various genetic factors, epigenetic factors and the imbalance in immune regulation can significantly contribute to the development of this disease. In addition, the experimental autoimmune uveitis (EAU) has been commonly used to further explore the pathogenesis of the disease. Herein, in this review article, we discuss about the major research advances made in understanding of the different epigenetic factors and gut microbes involved in the pathogenesis of VKH syndrome as well as EAU. The information discussed can help to better understand the pathogenesis of VKH syndrome, and thereby might provide a basis for finding novel molecular targets and innovative treatment strategies in the future.

Iris Manifestations in Inadequately Treated Chronic Recurrent Vogt-Koyanagi-Harada Disease.

PURPOSE: To describe the iris changes in chronic recurrent Vogt-Koyanagi-Harada Disease (VKH). METHODS: Retrospective case series. Clinical features of 3 VKH patients who developed bilateral depigmentation of the iris are described. RESULTS: Patients had delayed diagnosis and inadequately treated chronic recurrent VKH. Patient 1 presented with bilateral multiple contiguous small granulomatous iris stromal lesions, severe diffuse iris swelling more severe near the iris root, producing peripheral iris undulations. Diffuse iris atrophy and peripheral depigmentation subsequently developed. Patient 2 presented with peripheral iris depigmentation, atrophy of the ciliary zone iris stroma and sparing of the sphincter pupillae, giving a 'sunflower appearance.' Patient 3 had extensive iris depigmentation, atrophy, and transillumination defects involving the entire iris. CONCLUSION: Uncontrolled bilateral diffuse granulomatous stromal iris inflammation leads to subsequent depigmentation, transillumination defects and atrophy, beginning in the peripheral iris. Sphincter pupillae is initially spared but complete iris atrophy may develop.

A case presentation of an IgA nephropathy patient with Vogt-Koyanagi-Harada syndrome.

BACKGROUND: Vogt-Koyanagi-Harada syndrome is a rare disease characterized by skin and eyelash bleaching, chronic granulomatous iridocyclitis and exudative retinal detachment, and aseptic meningitis and encephalopathy. IgA nephropathy complicated by Vogt-Koyanagi-Harada syndrome is very rare, even though they might have similar pathogeneses. Ocular lesions often are not examined when patients are diagnosed with IgA nephropathy, which affects the prognosis. CASE PRESENTATION: We describe a 55-year-old male IgA nephropathy patient who was admitted with high fever and hematuria. Physical examination revealed impaired binocular vision with blurred vision, impaired hearing, and a congestive rash on the chest and back. Renal ultrasound examination showed no abnormalities. Laboratory examination showed that glomerulonephritis was complicated by infection, and anti-infection therapy was ineffective. Bilateral fluorescein angiography showed Vogt-Koyanagi-Harada syndrome. Further renal biopsy confirmed IgA nephropathy. Hormone shock therapy and cyclophosphamide adjuvant therapy were administered, and the patient's symptoms improved. CONCLUSION: For the first time, we reported the case of simultaneous onset of IgA nephropathy and Vogt-Koyanagi-Harada syndrome, which is very rare. The onset of Vogt-Koyanagi-Harada syndrome is rapid and serious, while that of IgA nephropathy is relatively milder, making it easy for specialized doctors to neglect this condition. Doctors should be highly alert to the clinical concomitant occurrence of the two diseases with similar mechanisms, especially in the case of neurological defects and ocular symptoms in IgA nephropathy patients, since timely immunosuppressive treatment may improve the outcome of ocular diseases.

Increased Expression of Indoleamine 2,3-Dioxygenase (IDO) in Vogt-Koyanagi-Harada (VKH) Disease May Lead to a Shift of T Cell Responses Toward a Treg Population.

Previous studies have pointed out that indoleamine 2,3-dioxygenase (IDO), the rate-limiting enzyme initiating tryptophan metabolism, plays a role in the regulation of the immune system. This project was designed to investigate the potential role of IDO in monocyte-derived dendritic cells (moDCs) obtained from active Vogt-Koyanagi-Harada (VKH) disease patients. In this study, we found that the IDO mRNA expression and enzyme activity were increased in active VKH patients as compared with healthy controls and patients in remission. To investigate the role of IDO in immune regulation, an effective inhibitor 1-methyl-L-tryptophan (1-MT) was used to suppress its activity in DCs. The results showed that inhibition of IDO with 1-MT significantly decreased the expression of DC marker CD86. IDO inhibition did not affect the cytokine production of IL-6, IL-1ß, TNF-α, IL-10, and TGF-ß in DCs. Downregulation of IDO in DCs also led to the reduction of regulatory T (Treg) cells and an increased CD4+ T cell proliferation. Treatment with 1-MT did not affect the phosphorylation of the MAPK pathway in DCs. In general, our study suggests that IDO may play an important role in the pathogenesis of VKH disease by regulating DC and CD4+ T cell function. Tryptophan deficiency and kynurenine accumulation may account for the complicated effects of IDO. Further research is needed to study the precise tryptophan metabolites that may limit immune responses in VKH disease.

Decreased Expression of TGR5 in Vogt-Koyanagi-Harada (VKH) Disease.

Purpose: To investigate the role of G-protein-coupled bile acid receptor-1, Gpbar1 (TGR5) in the pathogenesis of Vogt-Koyanagi-Harada (VKH) disease.Methods: The mRNA level of TGR5, iNOS, Arg1, CD16, and CD206 in macrophages was assayed by real-time PCR. ELISA was used to detect the production of cytokines in cell culture supernatants. The frequencies of CD4+IFN-γ+ and CD4+ IL-17+ T cells were tested by flow cytometry.Results: A decreased expression of TGR5 in M1 macrophages was observed in active VKH patients as compared with normal controls. TGR5 stimulation of M1 macrophages with INT-777 caused a shift of the inflammatory M1 toward the anti-inflammatory M2 macrophage subtype. TGR5 activation of macrophages co-cultured with CD4+ T cells inhibited Th1 and Th17 polarization, as well as the release of IFN-γ and IL-17 in the culture supernatant.Conclusion: Our results show that a decreased TGR5 expression might contribute to the pathogenesis of VKH disease.

Lord Nelson's (1758-1805) left eye.

Following the loss of his right eye at Calvi in 1794, Lord Nelson suffered increasing left-sided visual loss, here considered most likely to have been due to the ocular inflammatory condition 'sympathetic ophthalmia'. It is also argued that his succeeding episodes of violent headaches with nausea and prostration, and possible depigmentation of hair, reflected the development of an uveomeningoencephalitic syndrome akin to that of Vogt-Koyanagi-Harada disease, which is best regarded as the same condition with a different aetiology.

Case Series: Gene Expression Analysis in Canine Vogt-Koyanagi-Harada/Uveodermatologic Syndrome and Vitiligo Reveals Conserved Immunopathogenesis Pathways Between Dog and Human Autoimmune Pigmentary Disorders.

Vogt-Koyanagi-Harada syndrome (VKH) and vitiligo are autoimmune diseases that target melanocytes. VKH affects several organs such as the skin, hair follicle, eyes, ears, and meninges, whereas vitiligo is often limited to the skin and mucosa. Many studies have identified immune genes, pathways and cells that drive the pathogeneses of VKH and vitiligo, including interleukins, chemokines, cytotoxic T-cells, and other leukocytes. Here, we present case studies of 2 canines with VKH and 1 with vitiligo, which occurred spontaneously in client-owned companion dogs. We performed comparative transcriptomics and immunohistochemistry studies on lesional skin biopsies from these cases in order to determine if the immunopathogenesis of autoimmune responses against melanocytes are conserved. In dogs, we found enrichment of T cell gene signatures, with upregulation of IFNG, TNF, PRF1, IL15, CTSW, CXCL10, and CCL5 in both VKH and vitiligo in dogs compared to healthy controls. Similar findings were reported in humans, suggesting that these genes play a role in the pathogenesis of spontaneous VKH and vitiligo. T cell-associated genes, including FOXP3 and TBX21, were enriched, while IGFBP5, FOXO1, and PECAM1 were decreased compared to healthy controls. Further, we identified TGFB3, SFRP2, and CXCL7 as additional potential drivers of autoimmune pigmentary disorders. Future studies exploring the immunopathogenesis of spontaneous autoimmunity will expand our understanding of these disorders, and will be useful in developing targeted therapies, repurposing drugs for veterinary and human medicine, and predicting disease prognosis and treatment response.

Aqueous cytokine levels in four common uveitis entities.

To investigate aqueous cytokine profiles in acute anterior uveitis (AAU), Fuchs' syndrome, Vogt-Koyanagi-Harada (VKH) disease and Behcet's disease (BD), we assayed the concentrations of 17 cytokines by multiplex immunoassay in aqueous humor (AqH) collected during cataract surgery from 24 AAU, 29 Fuchs' syndrome, 29 VKH disease, 30 BD and 30 senile cataract control patients. Aqueous cytokine levels were compared between the five groups and analysed by logistic regression. Cytokine levels were then compared between uveitis patients who underwent cataract surgery within 3 months and those who underwent this surgery more than 3 months after complete control of intraocular inflammation. The results showed that aqueous levels of interferon (IFN)-γ, monocyte chemotactic protein (MCP)-1, macrophage inflammatory protein (MIP)-1ß and tumour necrosis factor (TNF)-α levels in AqH from patients with Fuchs' syndrome were significantly higher than those in the other four groups. Using multivariate analysis, MIP-1ß was found to be significantly associated with Fuchs' syndrome. There was no difference in aqueous cytokine levels between cases having cataract surgery within 3 months compared to those after 3 months of complete control of their intraocular inflammation. The current study shows that Chinese patients with Fuchs' syndrome appear to have a specific cytokine profile. MIP-1ß is an important chemokine in the intraocular environment of Fuchs' syndrome. Aqueous cytokine profiles support the performance of cataract surgery in uveitis within 3 months after intraocular inflammation control.

Replication of Genome-Wide Association Analysis Identifies New Susceptibility Loci at Long Noncoding RNA Regions for Vogt-Koyanagi-Harada Disease.

Purpose: This study was aimed at investigating the association of long noncoding RNA (lncRNA)-related single nucleotide polymorphisms (SNPs) with Vogt-Koyanagi-Harada (VKH) disease. Methods: LncRNA-related SNPs were selected by multi-omics analysis. Genotyping, expression of lncRNA and mRNA, cell proliferation, and cytokine production were tested by MassARRAY System, real-time PCR, CCK8, and ELISA. Results: A significant association with VKH was found for lnc-TOR3A-1:1/rs3829794, which is located in a non-HLA region (CC genotype: Bonferroni corrected P values [PC] = 2.98 × 10-8, odds ratio [OR] = 0.62; TT genotype: PC = 1.64 × 10-8, OR = 1.57; C allele: PC = 1.39 × 10-12, OR = 0.71). Additionally, an association was found for four lncRNA SNPs located in the HLA region. Functional experiments in rs3829794 genotyped individuals showed decreased ABL2 (ABL proto-oncogene 2, nonreceptor tyrosine kinase) expression, decreased proliferation of anti-CD3 plus anti-CD28-stimulated peripheral blood mononuclear cells (PBMCs), and an increased production of IL-10 in CC carriers compared to TT carriers (P = 0.0073, P = 0.0011, and P = 0.002, respectively). Conclusions: Our study identified five new loci associated with VKH susceptibility and identified a functional variant (lnc-TOR3A-1:1/rs3829794) that confers risk for VKH, which is possibly mediated by modulating gene expression, proliferation of lymphocytes, and regulation of anti-inflammatory cytokine production.

Macular Abnormalities in Vogt-Koyanagi-Harada Disease.

Purpose: To investigate the prevalence of macular abnormalities in Chinese Vogt-Koyanagi-Harada (VKH) patients.Methods: Clinical characteristics, therapeutic effectiveness and visual outcome were reviewed and analyzed.Results: The most common macular abnormality was macular edema (ME), followed by macular choroidal neovascularization (CNV). Macular abnormalities were associated with recurrent episodes, disease course and visual acuity ≤20/50 at first visit. The prevalence of macular abnormalities in patients who were not treated according to our regular treatment regimen with corticosteroids combined with immunosuppressive agents and who were followed-up for at least one year (13.1%) was significantly higher than in patients receiving the regular treatment (5.7%). Visual improvement was found in 66.7% of eyes with macular abnormalities after regular treatment.Conclusion: Macular abnormalities were associated with recurrent uveitis, course of disease and lower visual acuity at first visit. Regular treatment could prevent the development of macular abnormalities and improved visual outcome in most patients.

Bilateral Cochlear Implantation in Vogt-Koyanagi-Harada Syndrome: A Case Report.

OBJECTIVE: To evaluate cochlear implantation in Vogt-Koyanagi-Harada syndrome with regard to surgical difficulties and hearing outcomes. PATIENTS: Single case report. INTERVENTION(S): Therapeutic complete electrode insertion during surgery despite evident intra-cochlear fibrosis. MAIN OUTCOME MEASURE(S): Postoperative speech audiometry, categories of auditory performance, and speech intelligibility rating. RESULTS: Vogt-Koyanagi-Harada syndrome is a multisystem autoimmune disease that affects tissues containing melanin. It is characterized by bilateral uveitis with auditory, vestibular, and dermatologic manifestations. Standard treatment comprises aggressive administration of systemic corticosteroids.This report describes the use of bilateral cochlear implants in a 30-year-old Saudi woman who presented with uveitis and was diagnosed with Vogt-Koyanagi-Harada syndrome. She had progressive hearing loss in both ears and experienced minimal improvement with hearing aids. The patient underwent sequential cochlear implantation, which was challenging because it was difficult to insert the electrode in the right ear due to intra-cochlear fibrosis. After more than 5 years of follow-up, she has good hearing in both ears, despite the advancement of ocular disease and recurring visual complaints. CONCLUSION: This study suggests that patients with Vogt-Koyanagi-Harada syndrome could develop intra-cochlear fibrosis during cochlear implantation due to the autoimmune nature of disease. Moreover, cochlear implantation becomes more difficult with disease advancement. Therefore, it is essential that healthcare professionals consider early detection and prompt treatment of hearing loss in patients with this syndrome.

Vogt-Koyanagi-Harada syndrome presenting with bilateral optic disc swelling and leptomeningeal enhancement.

Vogt-Koynagi-Harada (VKH) disease is a clinical syndrome with classical ocular and extraocular findings that is not uncommonly described in Asian, Middle-Eastern and South American populations. We describe a case of VKH in an elderly Polish-Australian distinguished by prominent bilateral disc swelling rather than uveitis and marked leptomeningeal enhancement on MRI which led to extensive investigation including brain biopsy. Both disc oedema and MRI abnormalities improved dramatically with systemic steroid therapy. VKH disease is an important differential to consider in older patients with an uveo-meningeal picture and atypical eye findings where other causes have been excluded.

Choroidal Vasculature from Ultra-Widefield Images without Contrast Dye and Its Application to Vogt-Koyanagi-Harada Disease.

PURPOSE: To develop a method to obtain ultra-widefield choroidal vessel images with a fundus camera without using dye, and its application in Vogt-Koyanagi-Harada (VKH) disease. DESIGN: Experimental study and case series. PARTICIPANTS: Patients with unilateral retinal disorders and those with VKH disease who had undergone ultra-widefield imaging were studied. METHODS: Indocyanine green angiography (ICGA) and the 635-nm wavelength Optos ultra-widefield fundus photography (Optos, United Kingdom) images (Optos635-nm) were processed by KagoEye 3 software. The 2 types of images were overlapped. The degree of overlapped blood vessel areas of the ICGA image was taken as the matching ratio. The matching ratio was obtained for the peripheral, posterior pole, and overall areas. In addition, changes in the fundus findings were followed up with Optos635-nm image analysis in patients with VKH disease. The degree of visibility of the choroidal vessels was evaluated for 5 stages. The clarity scores and the longitudinal findings were compared. MAIN OUTCOME MEASURES: The matching ratios between the ICGA images and the Optos635-nm images processed by KagoEye3 software were determined. RESULTS: Initially, 10 healthy eyes were studied. The matching ratios for the overall area, the peripheral area, and the posterior pole area of the ICGA image and the Optos635-nm image were 64.09%, 74%, and 63.10%, respectively. The correlations between the choroidal blood vessel matching ratio and the ocular axial length and refractive error were not significant, but the matching ratio was correlated significantly with the age. The average clarity score in 12 VKH disease patients was 1.6 ± 0.85 before treatment, which was significantly improved to 4.2 ± 0.75 after 1 month (P < 0.05). Many hyporeflective spotty lesions were observed on the Optos635-nm images, which coincided with hyperfluorescent dots on the ICGA images. The lesions gradually disappeared and the vortex vein became visible after treatment. CONCLUSIONS: The ultra-widefield Optos635-nm images processed by KagoEye3 software can exaggerate images of the choroidal vessels in widefield fundus images without using dye. Because this method is noninvasive, it is applicable to a variety of diseased and healthy eyes.

Unique clinical spectrum with distinguishing diagnostic features in Vogt-Koyanagi-Harada syndrome.

A 36-year-old ulcerative colitis male patient on treatment for 7 years was referred to dermatology with resistant alopecia universalis and hypopigmented patches on limbs for 5 months. During this time he also reported to ophthalmology with acute bilateral decreased vision for 5 days. His examination revealed hyperaemic discs, multifocal retinal detachments and choroidal granulomas. Taking into account the revised diagnostic criteria, atypical course of disease in the form of early cutaneous presentation followed by ophthalmic manifestations was attributed to Vogt-Koyanagi-Harada syndrome (VKHS) which was supported by relevant investigations including ophthalmic imaging, MRI and nerve conduction studies. Subclinical nerve conduction abnormalities and white matter demyelination were also seen for the first time in a patient of VKHS. Appropriate treatment was required to prevent visual complications; therefore, systemic corticosteroids with steroid sparing immunosuppressive drug therapy showed significant improvement in vision on follow-up. Cutaneous manifestations were resilient to the entire regimen.

Disabled-2 (DAB2) Overexpression Inhibits Monocyte-Derived Dendritic Cells' Function in Vogt-Koyanagi-Harada Disease.

Purpose: Recent studies reported that the tumor suppressor disabled-2 (DAB2) is a negative regulator of immune function. In this study, we investigated the role of DAB2 in monocyte-derived dendritic cells (DCs) from Vogt-Koyanagi-Harada disease (VKH) patients. Methods: The mRNA and protein levels of DAB2 were quantified by quantitative real-time PCR and Western blot. The Sequenom MassARRAY system was used to detect the promoter methylation level. An adenovirus carrying the DAB2 gene was transduced into immature DCs, isolated, and induced from active VKH patients. The surface markers of DCs, the frequency of T helper (Th) type 1 (Th1) and Th17 cells in CD4+T cells, which were cocultured with DCs, were tested by flow cytometry. ELISA was used to analyze the inflammatory cytokines produced by DC and CD4+T cell cocultures. Results: The mRNA and protein expression levels of DAB2 in DCs obtained from active VKH patients were decreased, while the DAB2 promoter methylation level was marginally increased when compared with inactive VKH patients and normal controls. The expression of CD86 on DCs was significantly downregulated by DAB2 overexpression. The DC-related inflammatory factors IL-6 and TNF-α were also decreased. The frequency of Th1 and Th17 cells and their related cytokines were reduced significantly after coculture with DAB2 overexpressing DCs. DAB2 overexpression did not affect autophagy in DCs from VKH patients. Conclusions: These results suggest that the decreased expression of DAB2 in DCs plays a role in the pathogenesis of VKH disease. DAB2 overexpression inhibits DC function, but this is not mediated via autophagy.

Optical Coherence Tomography Angiography for Evaluation of Sattler's Layer in Vogt-Koyanagi-Harada Disease.

The authors report a case of acute Vogt-Koyanagi-Harada syndrome with massively impaired perfusion in the Sattler's layer on optical coherence tomography angiography (OCTA). The hypoperfusion fully resolved during a period of 4 weeks and correlated well with hypofluorescent spots on indocyanine green angiography. This is the first time reduced perfusion in Sattler's layer has been observed on OCTA. This finding may aid further understanding of the pathology underlying VKH syndrome and indicates that evaluation of deeper choroidal layers like Sattler's layer may be of importance for disease monitoring and predicting prognosis in choroidal diseases. [Ophthalmic Surg Lasers Imaging Retina. 2018;49:639-642.].