[Recurrent chronic Vogt-Koyanagi-Harada syndrome or sarcoidosis? A Gloomy diagnosis]. / ¿Síndrome de Vogt-Koyanagi-Harada crónico o sarcoidosis? Un diagnóstico sombrío.

Background: Vogt-Koyanagi-Harada syndrome (VKH) is a systemic disease that affects organs profuse in melanocytes, presenting with a chronic and diffuse bilateral granulomatous panuveitis, as well as neurological, auditory, and cutaneous manifestations. In this article, a systematic approach is presented for the diagnostic management of VKH syndrome, considering relevant diagnostic possibilities to rule out other entities that manifest similar symptoms. Clinical case: 71-year-old man with a long-standing history of vitiligo, who experienced visual loss in his right eye 6 months before his admission, along with bilateral hearing loss predominantly in the right ear. During his hospitalization, he presented with chronic headache, fever, and significant involuntary weight loss. Ophthalmological examination revealed that his right eye only perceived light and had hyperemic bulbar conjunctiva, while the left eye had a visual acuity of 20/200. The right fundus had scattered pigmentation, while the left had an edematous optic disc and right optic atrophy. Conclusions: The diagnosis of VKH syndrome is established by the presence of the 5 diagnostic criteria for complete disease, including retinal depigmentation, neurological alterations, and dermatological manifestations. Since patients can present with a wide variety of symptoms, initial differential diagnosis should be considered, which represents a diagnostic challenge.

Introducción: el síndrome de Vogt-Koyanagi-Harada (VKH) es una enfermedad sistémica que afecta a órganos ricos en melanocitos y se manifiesta con una panuveítis granulomatosa bilateral, crónica y difusa, así como con manifestaciones neurológicas, auditivas y cutáneas. En este artículo se presenta un enfoque sistemático para el abordaje diagnóstico del síndrome de VKH y se consideran las posibilidades diagnósticas relevantes para descartar otras entidades que se presentan con síntomas similares. Caso clínico: hombre de 71 años con antecedentes de vitiligo de larga data, quien experimentó una pérdida visual en su ojo derecho seis meses antes de su ingreso, junto con hipoacusia bilateral, predominantemente en el oído derecho. Durante su hospitalización, presentó cefalea crónica, fiebre y una significativa pérdida involuntaria de peso. En la exploración oftalmológica, el ojo derecho solo percibía luz y presentaba conjuntiva bulbar hiperémica, mientras que el ojo izquierdo tenía una agudeza visual de 20/200. El fondo del ojo derecho presentaba pigmentación dispersa, mientras que el izquierdo tenía una papila edematosa y atrofia óptica derecha. Conclusiones: el diagnóstico del síndrome de VKH se establece mediante la presencia de los 5 criterios diagnósticos para la enfermedad completa, incluida la hipopigmentación retiniana, las alteraciones neurológicas y las manifestaciones dermatológicas. Dado que los pacientes pueden presentar una amplia variedad de síntomas, el diagnóstico diferencial debe considerarse inicialmente, lo que representa un desafío diagnóstico.

Síndrome de Vogt-Koyanagi-Harada / Vogt-Koyanagi-Harada Syndrome

El síndrome de Vogt-Koyanagi-Harada es una enfermedad autoinmune multisistémica crónica, caracterizada por panuveítis difusa granulomatosa bilateral con desprendimiento exudativo de retina y papilitis. Compromete el sistema nervioso central (meninges, disacusia neurosensorial) así como piel y mucosas. A pesar de ser una enfermedad compleja y poco frecuente, se hace necesario comprender la importancia del diagnóstico rápido y el tratamiento oportuno con seguimiento especializado. Es por ello que se decidió realizar una revisión de la literatura con el objetivo de actualizar los conocimientos existentes sobre este tema. La búsqueda se realizó en diferentes publicaciones y textos básicos de la especialidad. Las fuentes consultadas fueron las bases de datos PubMed y Google Scholar. El diagnóstico de la enfermedad es esencialmente clínico y son los oftalmólogos quienes más lo sospechan por ser los síntomas oculares los más frecuentes y dramáticos. El pronóstico visual de los pacientes es generalmente bueno si el diagnóstico es precoz y se indica un tratamiento adecuado. Los corticosteroides sistémicos a altas dosis asociados a inmunosupresores y agentes biológicos tienen gran impacto en la evolución de la enfermedad, sobre todo estos últimos a nivel mundial, previniendo complicaciones y permitiendo resultados visuales satisfactorios para una mejor calidad de vida del paciente(AU)

Vogt-Koyanagi-Harada syndrome is a chronic multisystem autoimmune disease characterized by bilateral diffuse granulomatous panuveitis with exudative retinal detachment and papillitis. It involves the central nervous system (meninges, sensorineural dysacusis) as well as skin and mucous membranes. In spite of being a complex and infrequent disease, it is necessary to understand the importance of rapid diagnosis and timely treatment with specialized follow-up. For this reason, it was decided to carry out a review of the literature with the aim of updating the existing knowledge on this subject. The search was carried out in different publications and basic texts of the specialty. The sources consulted were the PubMed and Google Scholar databases. The diagnosis of the disease is essentially clinical and it is the ophthalmologists who suspect it the most because the ocular symptoms are the most frequent and dramatic. The visual prognosis of patients is generally good if the diagnosis is early and adequate treatment is indicated. Systemic corticosteroids at high doses associated with immunosuppressants and biological agents have a great impact on the evolution of the disease, especially the latter worldwide, preventing complications and allowing satisfactory visual results for a better quality of life of the patient(AU)

Regarding Kaya P et al. "Predictive Factors for the Prognosis of Vogt-Koyanagi-Harada Disease".

INTRODUCTION: VKH is a primary stromal choroiditis. Studies with indocyanine green angiography (ICGA) have shown that inflammation begins at the choroid and may persist without clinically apparent inflammation. Thus, systemic therapy must target choroidal inflammation and be maintained until the choroiditis resolves, as proven by ICG and/or EDI-OCT imaging studies. DISCUSSION: Aggressive therapy with oral corticosteroids, with or without "pulsed" intravenous methylprednisolone, has been the mainstay of VKH therapy in the acute phase for many years. However, there is convincing evidence that corticosteroid monotherapy is insufficient to prevent progression onto the chronic recurrent form of the disease, which is associated with poor visual outcomes. CONCLUSION: Numerous studies suggest that combined therapy with systemic corticosteroids and immunosuppressants is necessary to prevent the progression toward chronic recurrent disease, avoiding complications, get a better control of inflammation and visual outcomes in VKH patients seen at the uveitic phase of the disease.

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

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.

Possible Association between Vogt-Koyanagi-Harada Disease and Coronavirus Disease Vaccine: A Report of Four Cases.

Vogt-Koyanagi-Harada (VKH) syndrome is an autoimmune, multisystemic disease characterized by severe bilateral granulomatous posterior, which can occur due to viral infection or vaccination. We report four cases that had a likely association between VKH disease and severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) vaccination. Two patients had VKH symptoms within 1 and 2 weeks after receiving the ChAdOx1 nCoV-19 vaccine. One patient presented with VKH symptoms 5 days after receiving the BNT162b2 vaccine, and one patient had symptoms within 4 weeks after receiving the CoronaVac vaccine. Early diagnosis and treatment of VKH disease are essential for the visual prognosis of this aggressive disease. Further in-depth studies are necessary to investigate this likely association to enable ophthalmologists to identify new assumed correlations between the diseases described in this study.

Risk factors for secondary Glaucoma in patients with Vogt-Koyanagi-Harada disease.

BACKGROUND/PURPOSE: Identify the prevalence and risk factors for secondary glaucoma among Mexican-mestizo patients with Vogt-Koyanagi-Harada Disease (VKH). METHODS: Retrospective cohort study analyzing the demographic, clinical, and epidemiological variables. Risk estimates were calculated using a Cox proportional hazards regression model. RESULTS: One hundred eyes of 50 patients, 44 (88%) women and 6 men (12%) with a median age of 35.5 years (IQR 29-46) and a median follow-up time of 72 months (IQR 13.7-126.7) were analyzed. The prevalence of glaucoma was 20%, with angle-closure accounting for 70% of all cases. Significant clinical risk factors for glaucoma development were a chronic recurrent stage at presentation (RR 2.88, 95% CI 1.11-12.63, p = 0.037), ≥ 2 episodes of recurrent anterior uveitis (RR 8.52, 95% CI 2.02-35.92, p < 0.001), angle-closure disease (ACD, RR 7.08, 95% CI 2.44-20.48, p < 0.001), iris bombé (RR 5.0, 95% CI 2.10-11.90, p < 0.001), and peripapillary atrophy (RR 3.56, 95% CI 1.43-8.85, p < 0.001). Exposure to > 24 months of oral (RR 9.33, 95% CI 2.21-39.28, p < 0.001) or > 12 months of topical corticosteroids (RR 3.88, 95% CI 1.31-11.46, p = 0.007) were associated with an increased likelihood for secondary glaucoma development. CONCLUSION: Glaucoma is a frequent complication of VKH, often attributed to mixed pathogenic mechanisms. Chronic disease at presentation, recurrent inflammation, angle-closure mechanisms, iris bombé, and peripapillary atrophy represent clinically significant risk factors for developing secondary glaucoma. Prompt and aggressive steroid-spearing immunosuppressive therapy for adequate inflammation control may lower the risk of glaucoma in VKH.

Vogt-Koyanagi-Harada disease: the step-by-step approach to a better understanding of clinicopathology, immunopathology, diagnosis, and management: a brief review.

BACKGROUND: Appraisals of Vogt-Koyanagi-Harada disease (VKH) have become progressively more complete, since its first description in 1906. The availability of new investigational methods has improved our knowledge of the immunopathology, clinicopathology, diagnosis, and management of VKH disease. This review aimed to describe some of the steps that led to better characterization of VKH as a clinical entity. METHODS: We searched on PubMed for articles that described the history of VKH disease and analyzed the progress in disease appraisal with new investigational and imaging methods. In particular, we searched for articles that investigated the clinicopathology, diagnosis, and management of VKH. FINDINGS: The following developments were considered essential for improving the appraisal and understanding of VKH: (1) the history of the disease, (2) immunopathological mechanisms, (3) clinicopathology, (4) the importance of distinguishing initial-onset from chronic disease, (5) relevant imaging modalities, among which indocyanine green angiography is crucial, (6) diagnostic criteria that facilitate early diagnosis, and (7) the need for early, prolonged, aggressive treatment that combines steroidal and non-steroidal immunosuppression. CONCLUSION: Based on these findings, the definition of VKH has improved. VKH disease starts in the choroidal stroma and later involves other structures when it is not diagnosed and treated early. Indocyanine green angiography and enhanced depth imaging optical coherence tomography facilitate early diagnosis and precise monitoring of choroidal inflammation. ICGA is clearly the gold standard for appraisals and follow-ups in VKH disease, however EDI-OCT should be especially considered in those areas where ICGA is not fully available. These modalities have contributed substantially to a "cure" for VKH, when treatment is introduced within the therapeutic window of opportunity.

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

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.

Ocular Adverse Events following Yellow Fever Vaccination: A Case Series.

PURPOSE: To describe four cases of ocular adverse events resembling intraocular inflammatory and non-inflammatory conditions following yellow fever vaccination (YFV) during a recent yellow fever (YF) outbreak in Brazil. METHODS: Charts of patients diagnosed with ocular adverse events after YFV between January 2017 and January 2019 at two tertiary referral centers in Brazil. RESULTS: Four patients (two adults and two children) are reported. Case 1 presented with typical findings of central serous chorioretinopathy which resolved spontaneously; case 2 was diagnosed with acute Vogt-Koyanagi-Harada disease; cases 3 and 4 had bilateral diffuse retinal vasculitis. In the absence of infectious and noninfectious disorders, the temporal association between stand-alone YFV and onset of ocular symptoms within 15 days was interpreted as evidence of causation. CONCLUSIONS: Clinicians should be aware of the wide spectrum of possible ocular adverse reactions to stand-alone YFV.

Correlation between Subfoveal Choroidal Thickness and Anterior Segment Inflammation in Patients with Chronic Stage of Vogt-Koyanagi-Harada Disease.

PURPOSE: To correlate changes in subfoveal choroidal thickness (SCT) with the degree of anterior inflammatory activity in chronic Vogt-Koyanagi-Harada (VKH) disease. METHODS: Anterior segment inflammation was assessed using SUN anterior chamber cell grading criteria, and SCT was measured using EDI-OCT in patients with VKH at multiple visits. ANOVA was used to compare the mean SCT for each anterior chamber cell grade. Regression analysis was used to correlate the anterior segment cell grade and the SCT. RESULTS: 14 patients were included in the study. A total of 432 data points consisting of SCT and anterior segment cell values were analyzed. ANOVA demonstrated significant difference between the mean SCT for different anterior chamber cell grades (p < .0001). Regression analysis demonstrated significant correlation between SCT and grade of anterior chamber cells (R2 = 0.37, p < .001). CONCLUSIONS: Chronic VKH is characterized by a dynamic change in SCT that correlates with anterior segment inflammatory activity.

Risk factors for subretinal fibrosis in patients with Vogt Koyanagi Harada syndrome.

PURPOSE: To identify the risk factors for the development of subretinal fibrosis (SRF) among patients with Vogt-Koyanagi-Harada (VKH) syndrome. METHODS: In this case-control study, electronic clinical records from patients diagnosed with VKH syndrome who attended the Inflammatory Eye Disease Clinic at a tertiary care ophthalmology reference center were assessed to identify risk factors from demographic, clinical, and epidemiological variables. Cases were defined as SRF and VKH, whereas the controls were VKH patients without SRF. RESULTS: A total of 150 electronic charts were reviewed, 92 patients with a follow-up longer than 12 months were included; 39 cases and 53 controls. A multivariate analysis found bullous serous retinal detachment as a significant risk factor for SRF (adjusted OR 8.93, 95% CI 1.94-41.1). CONCLUSION: Patients with VKH syndrome who develop a bullous retinal detachment have an 8 times higher risk of developing SRF in the long term.

Vogt-Koyanagi-Harada Disease Following COVID-19 Infection.

A 29-year-old female presented to the emergency clinic with gradual visual disturbance in both eyes for 15 days duration, accompanied by bilateral tinnitus, and ocular pain that increased with ocular movements. One month prior to presentation, the patient had tested positive for severe acute respiratory syndrome coronavirus-2 but without complications. Visual acuity was 20/100 in the right eye and 20/300 in the left eye. Funduscopy demonstrated optic nerve swelling, radial nerve fiber striation disruption, and bilateral retinal folds. Optical coherence tomography showed serous (bacillary) retinal detachment and multifocal areas of hyper-reflective changes in the inner and outer plexiform layer with inner nuclear layer thickening and disruption of the interdigitation zone bilaterally. We present a case of incomplete Vogt-Koyanagi-Harada disease following COVID-19 infection.

Characteristics, Upon Presentation, of a Cohort of Patients with Vogt-Koyanagi-Harada Disease in Puerto Rico.

OBJECTIVE: To describe the clinical features, upon initial presentation, of a cohort of patients with Vogt-Koyanagi-Harada (VKH) disease who live in Puerto Rico. METHODS: A retrospective medical record review of patients with VKH was performed. The demographic and clinical characteristics were analyzed. RESULTS: Twenty-two patients who met the diagnostic criteria for VKH were identified and included in the analysis. The median age at presentation was 41 years; 68.2% were female, and all patients were Hispanics. Bilateral disease was present in 90.9% of patients, and 59.1% of patients were categorized as having probable VKH. A headache was reported in 54.5% of patients and was the most common complaint at the time of presentation; the second most common complaint was tinnitus, which was present in 22.7% of patients. Vitiligo, alopecia, and meningismus were each preset in 9.1% of patients while hearing loss and aseptic meningitis were each reported in 4.5% of patients. Seventy-seven percent of patients had either topical or systemic corticosteroid use prior to the initial encounter. CONCLUSION: Our study suggests that in Puerto Rico, patients with Vogt-Koyanagi-Harada disease may have a distinctive prevalence of characteristics at the presentation when compared to other ethnic groups, including other Hispanic cohorts.

Vogt-Koyanagi-Harada-like Disease following Yellow Fever Vaccination.

Purpose: To report the manifestation of Vogt-Koyanagi-Harada-like disease (VKH) following yellow fever vaccination.Methods: Case report.Results: A 34-year-old immunocompetent male had tinnitus, headache, and decreased vision after a booster dose of yellow fever vaccine. Visual acuity was 20/100 in the right eye and 20/80 in the left, with serous retinal detachment (SRD) and choroidal thickening identified on clinical examination and multimodal imaging. Lumbar puncture revealed pleocytosis and an increased protein content, but extensive investigations ruled out infectious/neurological diseases. Pulse intravenous methylprednisolone was given, followed by a tapering regimen of high-dose oral prednisone. Azathioprine was started early, 3 weeks after initiation of oral steroids. Intraocular inflammation and SRD rapidly resolved, with visual acuity reaching 20/20 in both eyes, after 3 weeks. No recurrence of intraocular inflammation or sign of depigmentation was so far noticed, at 2 years of follow-up.Conclusion: Yellow fever vaccine may be a possible trigger for VKH.

Outcomes of Intravitreal Bevacizumab in Choroidal Neovascularization in Vogt-Koyanagi-Harada Disease- A Prospective Study.

Purpose: To assess the effectiveness of intravitreal (IV) bevacizumab and the need for systemic immunosuppressive therapy (IMT) in choroidal neovascularization (CNV) in Vogt-Koyanagi-Harada disease (VKHD).Methods: CNV treatment consisted of three monthly IV bevacizumab injections as a loading dose; if intra/subretinal fluid (IRF) persisted, further injections were proceeded besides increment in systemic IMT. Outcome analyses at 3, 6, and 12 months were visual acuity, central foveal thickness, macular volume, IRF, and addition of IMT.Results: Seven eyes of six patients were included. Five patients (five eyes) completed a 12-month follow-up and received 12 IV bevacizumab injections. At the 12-month follow-up, visual acuity improved in four out of five eyes (p = .0568); all eyes had decreased macular volume (p = .0431) but they still had persistent IRF; and all cases needed IMT introduction/increment.Conclusion: Intravitreal bevacizumab in association with systemic IMT was effective for CNV in VKHD. Active CNV may indicate disease of inadequate clinical control.

Vogt-Koyanagi-Harada syndrome: a discussion about resistance to corticotherapy / Vogt-Koyanagi-Harada: uma discussão sobre resistência à corticoterapia

ABSTRACT Vogt-Koyanagi-Harada (VKH) syndrome is an inflammatory condition of unknown etiology that can affect the eye. The most common ocular manifestation related to VKH is bilateral diffuse uveitis associated to exudative retinal detachment. Although these patients respond well to steroid pulse therapy, we report a case of a 44-year-old female patient presenting bilateral exudative retinal detachment and clinical diagnosis of VKH, who did not respond to the first cycle of 3-day pulse therapy with methylprednisolone. The exudation was reabsorbed only after a second cycle of steroid therapy.

RESUMO A doença de Vogt-Koyanagi-Harada é inflamatória e de etiologia desconhecida, podendo afetar o olho. A manifestação ocular mais comum relacionada à doença de Vogt-Koyanagi-Harada é a uveíte difusa bilateral associada ao descolamento exsudativo da retina. Embora esses pacientes respondam bem à pulsoterapia com esteroides, relatamos um caso de paciente de 44 anos que apresentou descolamento exsudativo bilateral da retina com diagnóstico clínico de doença de Vogt-Koyanagi-Harada que não respondeu ao primeiro ciclo de pulsoterapia de 3 dias com metilprednisolona. A exsudação apenas reabsorveu após uma segunda rodada de terapia com esteroides.

Initial-onset acute and chronic recurrent stages are two distinctive courses of Vogt-Koyanagi-Harada disease.

PURPOSE: To describe distinctive stages of Vogt-Koyanagi-Harada (VKH) disease: initial-onset acute versus chronic recurrent disease. METHODS: A comprehensive literature review regarding stages and clinical presentations of VKH disease was conducted. RESULTS: Despite a list of signs that has been described as characteristic features of early or late phases of VKH disease, the current classification -developed by an international committee and published in 2001- does not consider a distinction regarding the time from onset of disease symptoms, and specific findings observed at certain time point from the symptoms presentation and outcomes related to the stage of VKH disease. In that sense, chronic recurrent VKH disease is more refractory to treatment and is associated with a higher rate of complications. Accordingly, this subset of VKH patients has poorer functional and anatomical outcomes than patients with an initial-onset acute disease. CONCLUSIONS: An early clear distinction of VKH phenotype [Initial-onset acute versus chronic recurrent disease] should be considered in each clinical scenario, evaluating the delay in diagnosis and the clinical presentation, since it may help clinicians to perform a correct disease prognosis categorization and thus to make treatment decisions in terms of potential refractoriness or expected clinical outcomes.

Vogt-Koyanagi-Harada Syndrome in Brazilian Children.

Purpose To evaluate the characteristics of Vogt-Koyanagi-Harada (VKH) syndrome in Brazilian children.Methods Clinical data were obtained from the medical records of six children with VKH disease from March 2014 to June 2018 at the Federal University of São Paulo, Brazil.Results Six patients met the diagnostic criteria for VKH. The patients, who ranged in age from 5 to 8 years, all presented with chronic disease. The most common ocular finding was abnormal fundus pigmentation in five patients followed by band keratopathy in four. Two patients had glaucoma, cataract, and subretinal neovascularization. The final visual acuity was less than 20/40 in 50% of the eyes.Conclusion Visual outcomes were favorable in half of patients depending on the long-term sequelae. VKH is rarely reported in children and it may be sight-threatening, and requires careful attention, being an important differential diagnosis.

Presumed atypical peripapillary Vogt-Koyanagi-Harada disease.

PURPOSE: To describe a case of bilateral presumed atypical Harada disease with sequential, not simultaneous, involvement of the peripapillary retina (subretinal fluid) in a healthy patient with no systemic complaints. OBSERVATION: A 35-year-old healthy white man presented with sudden paracentral visual loss in the left eye. His medical history was unremarkable. However, he reported a similar episode 20 months earlier in the right eye that was associated with macular serous retinal detachment. The right eye showed evidence of reactive peripapillary atrophy and pigmentary alteration in the macula. Optical coherence tomography scans of the posterior left eye segment revealed a diffuse thickened choroid, papillomacular subretinal exudate and discontinuity of the ellipsoid layer with suggestion of vitreous cellularity. Autofluorescence imaging of the left eye showed peripapillary hyperautofluorescence. A fluorescein angiogram revealed progressive staining and pooling of the peripapillary retina with corresponding retinal vasculitis. Indocyanine green angiography revealed multiple hypocyanescent lesions with an area of hypercyanescence temporal to the disc. Rheumatologic evaluation and laboratory tests were all negative. Chest tomography was normal. Considering the apparent absence of infectious diseases, the patient was started on 60 mg/day prednisone. After 8 days, visual acuity improved to 20/250, improving to 20/20 vision six months after a slow steroid wean. CONCLUSION: We believe our case represented a variant of the Vogt-Koyanagi-Harada syndrome in an atypical situation, because the patient fulfilled the presumed criteria. Furthermore, the findings of clinical and complementary examinations led to this nosological entity to the exclusion of others. IMPORTANCE: The point of this case is to alert ophthalmologists to the existence of this atypical presentation of the disease so that it should be included among the differential diagnoses of pathologies that present with these findings.

Full-field electroretinogram behavior in Vogt-Koyanagi-Harada disease: a 24-month longitudinal study in patients from acute onset evaluated with multimodal analysis.

PURPOSE: To prospectively evaluate the dynamic changes of the full-field electroretinogram (ff-ERG) and its association with inflammatory signs in patients with Vogt-Koyanagi-Harada disease (VKHD) followed up after acute onset. METHODS: Twelve acute VKHD patients, who were followed up for at least 24 months, were enrolled at a tertiary center from June 2011 to January 2017. Treatment consisted of intravenous methylprednisolone followed by 1 mg/kg/day of oral prednisone with a slow tapering associated with late non-steroidal immunosuppressive therapy in previously defined cases. Inflammation was systematically evaluated with clinical and posterior segment imaging (PSI) exams (fluorescein angiography, FA, indocyanine green angiography, ICGA, enhanced depth imaging optical coherence tomography, EDI-OCT). A ff-ERG was performed upon enrollment as well as at predefined intervals. Scotopic ff-ERG parameters changes between the 12th and 24th months defined the ERG-stable or ERG-worsening groups. "Flare" was defined as an appearance or worsening of inflammatory signs (after the initial 6 months following disease onset) under the predefined treatment protocol. RESULTS: ff-ERG parameters initially improved in all eyes; in the evaluation between the 12th and 24th months, ff-ERG results were stable in 17 eyes (71 %) and worsened in 7 eyes (29 %). Subnormal ff-ERG results were observed in 15 eyes (62 %) at the 24th month. On the other hand, the flare was observed in 8 eyes (33 %) as cells in the anterior chamber and in 24 eyes (100 %) as any PSI inflammatory sign. The ERG-worsening group presented thicker subfoveal choroid at the first month (p = 0.001) and fluctuations in choroidal thickness more often during follow-up when compared to the ERG-stable group (p = 0.02). CONCLUSIONS: Scotopic ff-ERG parameters worsened between the 12th and 24th months in a quarter of the patients. Subclinical inflammation detected as an increase in CT seems to be related to worsening in visual function measured with ffERG.