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El síndrome de Vogt-Koyanagi-Harada (VKH) es una rara enfermedad granulomatosa multisistémica caracterizada por aparición de panuveítis grave bilateral y desprendimiento seroso de retina; puede acompañarse de un amplio espectro de síntomas extraoculares como los auditivos, y la afección más frecuente es la hipoacusia neurosensorial. Su etiología se reconoce como respuesta autoinmune mediada por células T contra antígenos de melanocitos presentes en coroides, meninges, cóclea y piel. Asimismo, factores genéticos del huésped se han identificado como predisponentes para su aparición, y es la presencia del alelo HLA-DR4, en particular el subtipo HLA-DRB1 0405, el más estudiado hasta la fecha. El tratamiento se basa en administración de corticosteroides sistémicos en dosis altas, sin embargo, es escasa la evidencia que evalúa específicamente la eficacia de estos medicamentos sobre sus manifestaciones audiovestibulares. Este artículo expone un caso de síndrome de VKH con compromiso auditivo concomitante y realiza una breve revisión narrativa de la literatura.
Vogt-Koyanagi-Harada syndrome (VKHS) is a rare multisystemic granulomatous disease, characterized by severe bilateral panuveitis and serous retinal detachment; it can be associated with a wide spectrum of extraocular symptoms, such as auditory symptoms, and the most common condition is sensorineural hearing loss. Its etio-logy is recognized as a T-cell-mediated autoimmune response against melanocyte antigens present in the choroid, meninges, cochlea, and skin. Likewise, host genetic factors have been identified as predisposing for its development, specifically the pre-sence of the HLA-DR4 allele, the HLA-DRB1 0405 subtype is the most studied up to date. Treatment is based on the administration of high doses of systemic corticos-teroids, however, there is not much evidence that specifically evaluates the efficacy of these medications on their audiovestibular manifestations. This article presents a clinical case of VKH syndrome with concomitant hearing impairment and carries out a short narrative review of the literature.
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Humanos , Masculino , FemeninoRESUMEN
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.
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Sarcoidosis , Síndrome Uveomeningoencefálico , Masculino , Humanos , Anciano , Síndrome Uveomeningoencefálico/diagnóstico , Sarcoidosis/diagnóstico , Fondo de Ojo , Diagnóstico DiferencialRESUMEN
PURPOSE: We report the estimated incidence of, and risk factors for, recurrent anterior uveitis in patients with initial acute-onset Vogt-Koyanagi-Harada (VKH) disease using survival analyses. METHODS: Patients who were diagnosed with initial acute-onset VKH disease during 2003-2022 at two university hospitals were included. Recurrent anterior uveitis was defined as the first occurrence of the granulomatous anterior uveitis with anterior chamber cells and flare of 2+ or more by the Standardization of Uveitis Nomenclature (SUN) Working Group grading scheme, after the disappearance of conspicuous uveitis and serous retinal detachment for at least 3 months, regardless of systemic or local treatment. The univariate log-rank test and multivariate Cox regression analyses were performed, including patients' demographic characteristics, underlying diseases, presence of prodromal symptoms, duration of visual symptoms, visual acuity, slit-lamp and fundus findings, and height of serous retinal detachment. The treatment method and response to treatment were also included. RESULTS: The estimated incidence was 39.3% at 10 years. Fifteen of 55 patients (27.3%) had recurrent anterior uveitis during the mean follow-up of 4.5 years. The presence of focal posterior synechiae at the diagnosis increased the risk of recurrent anterior uveitis 6.97-fold compared to the absence of synechiae (95% confidence interval [CI], 2.20-22.11; p < 0.001). Use of systemic high-dose steroid therapy more than 7 days after the development of visual symptoms resulted in a hazard ratio of 4.55 (95% CI, 1.27-16.40; p = 0.020). CONCLUSIONS: This study reports the estimated incidence and risk factors of recurrent anterior uveitis in VKH disease from survival analyses. However, because of the retrospective nature of this study, it is hard to confirm the consistency of the medical records regarding risk factors, thus, the presence of focal posterior synechiae can be inconclusive as a risk factor. Further studies are warranted.
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Desprendimiento de Retina , Uveítis Anterior , Síndrome Uveomeningoencefálico , Humanos , Incidencia , Desprendimiento de Retina/diagnóstico , Desprendimiento de Retina/epidemiología , Desprendimiento de Retina/etiología , Estudios Retrospectivos , Factores de Riesgo , Uveítis , Uveítis Anterior/diagnóstico , Uveítis Anterior/epidemiología , Uveítis Anterior/tratamiento farmacológico , Síndrome Uveomeningoencefálico/complicaciones , Síndrome Uveomeningoencefálico/diagnóstico , Síndrome Uveomeningoencefálico/tratamiento farmacológico , RecurrenciaRESUMEN
Vogt-Koyanagi-Harada disease (VKHD) is a rare multisystemic granulomatous disorder that mainly affects the central nervous system, eyes, inner ears, and skin, basically organs rich with melanocytes. This case report describes an 11-year-old Saudi Arabian female who presented with a six-month history of decrease in vision in both eyes associated with neck pain, right ankle pain, fatigability, and skin depigmentation. Her ophthalmological examination showed visual acuity of 6/30 oculus dextrus (OD) and 6/60 oculus sinister (OS), and her fundoscopic examination revealed vitreous opacity mainly in the right eye. Optical coherence tomography (OCT) demonstrated macular edema along with infiltration and optic edema. She was initially diagnosed as having posterior uveitis and treated with oral prednisone and steroid eye drops. A month later, her ophthalmological examination revealed a rebound of macular edema. Dosages of steroid and adalimumab injection were raised, and azathioprine was added. Her left macular edema was not resolved; therefore, an aflibercept injection was added. A differential diagnosis of VKHD needs to be considered. Any patient who presents with posterior uveitis should be screened for VKHD. Physicians and ophthalmologists need to be more aware of VKHD, as it can cause serious complications.
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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.
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BACKGROUND: The tomographic finding, which has been called the "fingerprint sign" in en face reconstructions, seems to be the result of a variety of processes that cause distension of the outer plexiform layer (OPL) and the Henle fiber layer (HFL). The aim of this paper is to describe the appearance of concentric rings at the OPL/HFL interface visualized using en face reconstructions of cross-sectional optical coherence tomography images of patients with Vogt-Koyanagi-Harada disease. METHODS: Retrospective analysis of images of six eyes of three patients obtained by cross-sectional OCT imaging and en face reconstruction at the level of the OPL/HFL interface. RESULTS: All eyes presented with a dentate or saw-tooth pattern of the OPL/HFL interface on cross-sectional OCT with corresponding concentric rings on en face OCT reconstruction, consistent with the recently published "fingerprint sign". Initial OPL/HFL interface changes were observed between the first and fourth months after treatment and resolution of VKHD associated serous retinal detachments. These OPL/HFL interface changes have persisted for many years following the resolution of the active inflammation. CONCLUSIONS: Changes in the OPL/HFL interface can be identified following successful treatment of VKHD. These included both a dentate or saw-tooth pattern on cross-sectional imaging and concentric rings or the "fingerprint sign" on en face reconstructions. These changes persisted for many years despite disease quiescence.
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This report describes a patient who had acute lymphocytic leukemia with exudative retinal detachment (RD), which mimicked Vogt-Koyanagi-Harada disease (VKH). A 61-year-old woman presented with painless loss of vision in the left eye. Fundus examination revealed RD in her left eye. B-scan ultrasonography confirmed localized RD and choroidal thickening. Fundus fluorescein angiography revealed stippled pinpoint hyperfluorescence in the upper macula. One week later, reduced visual acuity was noted in the right eye. B-scan ultrasonography and optical coherence tomography revealed serous RD in both eyes. A provisional diagnosis of VKH was made. However, subsequent hematologic analysis detected an extremely high leukocyte count. Elevated numbers of leukocytes and tumor cells were found in cerebrospinal fluid. Bone marrow biopsy revealed 77% primary atypical blood cells, 89% of which were immature lymphocytes. The patient was subsequently diagnosed with acute lymphocytic leukemia and transferred to the Department of Hematology. However, the patient and her family refused chemotherapy; she eventually died. Our findings suggest that exudative RD, similar to VKH, could be a sign of leukemia. Pinpoint hyperfluorescence leakage is important for differential diagnosis, particularly with respect to VKH.
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Leucemia-Linfoma Linfoblástico de Células Precursoras , Desprendimiento de Retina , Síndrome Uveomeningoencefálico , Femenino , Angiografía con Fluoresceína , Humanos , Persona de Mediana Edad , Leucemia-Linfoma Linfoblástico de Células Precursoras/complicaciones , Leucemia-Linfoma Linfoblástico de Células Precursoras/diagnóstico por imagen , Leucemia-Linfoma Linfoblástico de Células Precursoras/tratamiento farmacológico , Desprendimiento de Retina/diagnóstico por imagen , Tomografía de Coherencia ÓpticaRESUMEN
Aims: To evaluate associations between vision-related (VR-) and health-related (HR-) QoL metrics and inflammation and treatment in non-acute VKHD patients.Methods: Cross-sectional study in a tertiary center in Sao Paulo, Brazil with 22 patients with non-acute VKHD followed prospectively for ≥12 months since acute disease onset, with systematic evaluation and predefined treatment protocols. VR- and HR-QoL aspects were assessed by VFQ-25 and SF-36 questionnaires, respectively. Associations between the questionnaire's subscale item scores with inflammation and systemic medical therapies were assessed.Results: After generalized linear model analysis, worse VA, severe fundus changes, fluctuation of VA and fluctuation of anterior chamber cells impacted negatively on VR-QoL items. Higher cumulative total dose of corticosteroids and use of immunosuppressive therapy impacted negatively on both questionnaires.Conclusion: Worse VA, clinical inflammation and systemic treatment have a significant impact on VR- and HR-QoL questionnaires. Subclinical choroidal inflammation did not seem to impact QoL.
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Glucocorticoides/uso terapéutico , Inmunosupresores/uso terapéutico , Inflamación/etiología , Calidad de Vida , Autoinforme , Síndrome Uveomeningoencefálico/tratamiento farmacológico , Agudeza Visual , Adulto , Anciano , Brasil/epidemiología , Estudios Transversales , Femenino , Estudios de Seguimiento , Humanos , Incidencia , Inflamación/diagnóstico , Inflamación/tratamiento farmacológico , Masculino , Persona de Mediana Edad , Estudios Prospectivos , Encuestas y Cuestionarios , Síndrome Uveomeningoencefálico/complicaciones , Síndrome Uveomeningoencefálico/epidemiología , Adulto JovenRESUMEN
PURPOSE: To evaluate associations between functional and structural measurements in patients with non-acute VKHD. METHODS: In this cross-sectional study, 16 non-acute VKHD patients (32 eyes; 14 female) were evaluated with multifocal electroretinogram (mfERG), standardized automated perimetry (SAP) and optical coherence tomography (OCT)examinations. All included patients had a minimum 12 months of follow-up from acute onset and were participants of an ongoing prospective study since acute phase with systematic clinical imaging evaluations and electroretinogram examinations within a predefined treatment. Age- and gender-matched controls were included. Main outcomes were functional and structural abnormalities and their correlation; secondary outcome was correlation of these findings with clinical characteristics, including fundus abnormalities. RESULTS: SAP and mfERG parameters were significantly worse in patients than in controls. Fourteen eyes (43.7%) had disrupted ellipsoid zone (EZ); visual acuity (VA) was similar between eyes with intact or disrupted EZ. Eyes with intact and disrupted EZ differed significantly concerning N1 and P1 amplitudes and N1 peak time values on mfERG and mean sensitivity (MS), central sensitivity (CS), foveal threshold, visual field index, mean deviation (MD) and pattern standard deviation values on SAP. The area under the curve on receiver operating curves for P1 amplitude was 0.81 (cut-off value = 34.7 nV/deg2 ) and for MD value was 0.84 (cut-off value = -5.2 dB). Central retinal thickness (CRT) significantly correlated with N1 and P1 amplitudes and P1 peak time values on mfERG (r = 0.354, r = 0.442 and r = -0.405, respectively) and MD, MS, CS and fovea threshold (log values) on SAP (r = 0.372, r = 0.406, r = 0.431 and r = 0.414, respectively). Statistically significant associations were found with the presence of peripapillary atrophy and recurrent anterior uveitis with a worse MD value (p = 0.004 and p < 0.001, respectively). CONCLUSION: In non-acute VKHD, disrupted EZ and reduced CRT were correlated with impaired mfERG and SAP parameters, even in patients with good VA.
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Electrorretinografía/métodos , Retina/fisiopatología , Tomografía de Coherencia Óptica/métodos , Síndrome Uveomeningoencefálico/diagnóstico , Agudeza Visual , Adulto , Estudios Transversales , Femenino , Humanos , Masculino , Estudios Prospectivos , Retina/diagnóstico por imagen , Síndrome Uveomeningoencefálico/fisiopatologíaRESUMEN
Este relato teve como objetivo apresentar um caso de hepatotoxicidade colestática induzida por azatioprina em portadora da síndrome de Vogt-Koyanagi-Harada. À admissão, apresentava icterícia +3/+4, acolia fecal e colúria, além de aumento de marcadores hepáticos, sendo compatível com síndrome colestática, cuja etiologia foi confirmada após exclusão de outras causas possíveis e retirada da azatioprina. A paciente evoluiu, após 1 semana de retirada do fármaco, com diurese livre de coloração menos escura e evacuação presente, sem acolia. Além disso, houve melhora nos exames que precederam a alta hospitalar
This report aimed at presenting a case of azathioprine-induced cholestatic hepatotoxicity in a patient with Vogt-Koyanagi-Harada syndrome. On admission, she presented with jaundice +3/+4, acholic feces, and choluria, as well as increased hepatic markers, all consistent with cholestatic syndrome, the etiology of which was confirmed after other possible causes were ruled out and azathioprine was discontinued. After 1 week of the drug discontinuation, the patient progressed with free diuresis of lighter color and defecation, with no acholia. In addition, tests performed before discharge were improved.
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Humanos , Femenino , Persona de Mediana Edad , Azatioprina/toxicidad , Azatioprina/uso terapéutico , Síndrome Uveomeningoencefálico/tratamiento farmacológico , Enfermedad Hepática Inducida por Sustancias y Drogas/diagnóstico , Inmunosupresores/toxicidad , Inmunosupresores/uso terapéutico , Sinusitis/tratamiento farmacológico , Azatioprina/efectos adversos , Tórax/diagnóstico por imagen , Radiografía , Colestasis Intrahepática/diagnóstico , Colestasis Intrahepática/sangre , Ultrasonografía , Neumonía Bacteriana/tratamiento farmacológico , Enfermedad Hepática Inducida por Sustancias y Drogas/sangre , Bocio Nodular/diagnóstico por imagen , Inmunosupresores/efectos adversos , Antibacterianos/uso terapéuticoRESUMEN
Objective: To assess structural changes in retina after systemic immunosuppressive treatment in Vogt-Koyanagi-Harada (VKH) disease using spectral-domain optical coherence tomography (SD-OCT). Methods: The clinical data of 17 VKH cases (34 eyes) who consecutively attended the Beijing Tongren Hospital between December 2015 and December 2019 were retrospectively reviewed. All the patients had acute or subacute onset, and underwent high-dose systemic corticosteroid and/or immunosuppressive treatment, with a followed-up time of at least 6 months. At the end of follow-up, the intraocular inflammation was controlled, and oral prednisone was withdrawn or being adjusted to less than 10 mg/day. The SD-OCT features of the included eyes were analyzed before treatment, 1 week, 1 month and 3 months after treatment, and at the last visit. Results: A total of 17 cases (34 eyes; 6 males and 11 females) were included, with an age of (42.2±10.6) years, and were followed up for (9.4±3.3) months. At 1 week after initiating treatment, the percentage of the eyes with extensive/multiple or multifocal retinal detachment in the macular region, membranous structures and intraretinal cysts, and undulations and bumps of retinal pigment epithelium (RPE) decreased (5.9% vs 100%, 2.9% vs 47.1%, 5.9% vs 70.6%, 11.8% vs 58.8%, respectively, all P<0.001). There was statistical significant difference of the percentage of ellipsoid zone and external limiting membrane (ELM) disruptions before treatment, at 3 months and the last visit (100% vs 35.3%, 64.7% vs 52.9%, 41.2% vs 26.5%, respectively, all P<0.001). At the last visit, there were statistical significant differences of the best corrected vision acuity (BCVA) between the intact ellipsoid zone group (20 eyes) and the discontinuous group (14 eyes), as well as between the intact ELM group (25 eyes) and the discontinuous group (9 eyes), respectively [0 (0, 0.05) vs 0.10 (0.03, 0.33) (log MAR), P=0.004; 0 (0, 0.07) vs 0.22 (0.05, 0.40) (log MAR), P=0.005]. Conclusions: The edema of choroid and retina subsided obviously at 1 week after initiating treatment. The extent of damage and recovery ability of ellipsoid zone and ELM were different before treatment, at 3 months and the last visit. At the last visit, the outer retinal layers failed to recover completely in some patients, and were related to the BCVA.
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Síndrome Uveomeningoencefálico , Enfermedad Aguda , Adulto , Femenino , Angiografía con Fluoresceína , Humanos , Masculino , Persona de Mediana Edad , Retina , Estudios Retrospectivos , Tomografía de Coherencia ÓpticaRESUMEN
Purpose: To assess health-related (HR-) and vision-related (VR-) quality of life (QoL) in patients with long-standing Vogt-Koyanagi-Harada disease (VKHD).Methods: Cross-sectional study of 49 patients with disease duration ≥12 months followed at Uveitis Service, Universidade de São Paulo, BR, for at least 12 months. HR- and VR-QoL were evaluated using SF-36 and NEI VFQ-25 questionnaires, respectively. Demographic, clinical and visual function data were compared with questionnaire scores.Results: After generalized linear models, lower mensal household income was associated with lower scores in both questionnaires while unemployment was associated with SF-36 questionnaire only. Treatment with peri-/intraocular medications and ocular surgery were associated with higher scores on SF-36 questionnaire. Worse visual acuity (VA), ocular complications and no ocular surgery were related to lower scores on NEI VFQ-25 questionnaire.Conclusions: On HR- and VR-QoL questionnaires difficulties perceived by patients with long-standing VKHD were mainly associated with socio-economic aspects, VA, local treatment and ocular complications.
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Estado de Salud , Encuestas Epidemiológicas/métodos , Calidad de Vida , Autoinforme , Síndrome Uveomeningoencefálico/psicología , Agudeza Visual , Adolescente , Adulto , Anciano , Segmento Anterior del Ojo/diagnóstico por imagen , Estudios Transversales , Electrorretinografía , Femenino , Angiografía con Fluoresceína , Estudios de Seguimiento , Fondo de Ojo , Humanos , Masculino , Persona de Mediana Edad , Oftalmoscopía , Estudios Retrospectivos , Índice de Severidad de la Enfermedad , Factores Socioeconómicos , Encuestas y Cuestionarios , Factores de Tiempo , Tomografía de Coherencia Óptica , Síndrome Uveomeningoencefálico/diagnóstico , Adulto JovenRESUMEN
RESUMO A síndrome de Vogt-Koyanagi-Harada (SVKH) é rara, multissistêmica e autoimune. Atinge principalmente os olhos, provocando uma panuveíte crônica bilateral, porém traz afecções em outras áreas e tecidos que são ricos em melanócitos, como olhos, orelha interna, meninges e a pele. Sua origem ainda não é totalmente conhecida. Geralmente, a SVKH atinge indivíduos de origem hispânica, do Oriente Médio, indianos, nativos americanos e asiáticos. Descrição dos aspectos audiológicos acometidos pela síndrome e as possíveis intervenções fonoaudiológicas para um caso específico. Paciente de 53 anos, sexo feminino, compareceu à Clínica de Fonoaudiologia, Faculdade de Odontologia de Bauru (FOB) com queixas audiológicas e diagnóstico médico da SVKH. A paciente apresentou perda auditiva sensório-neural bilateralmente, emissões otoacústicas evocadas ausentes e queixas vestibulares de vertigem postural e desequilíbrio ao andar, bem como queixa de zumbido agudo contínuo. O caso apresentado mostrou perda auditiva sensório-neural, vertigem, zumbido e acometimento ocular bilateral. Apesar do tratamento com corticoesteroide, a perda auditiva se manteve. Desta forma, precedente à indicação do AASI, o fonoaudiólogo deve atentar-se para o acompanhamento audiológico do caso, realização ou não de tratamento medicamentoso e ocorrência de sintomas sugestivos da síndrome, favorecendo o encaminhamento para o médico e participando ativamente do processo terapêutico envolvendo a audição.
ABSTRACT The Vogt-Koyanagi-Harada syndrome (VKHS) is a rare, multisystemic and autoimmune disease. The syndrome mainly affects the eyes, followed by bilateral chronic panuveitis, however, the syndrome may also affect the melanocytes tissues, for example, the eyes, inner ear, meninges and skin. The syndrome origin mechanism is not yet completely known. Commonly, the specific ethnic groups that are affected by the VKHS are as follows: Hispanics, Asians, Indians, Native Americans and ethnic groups from the Middle East. The audiological characteristics of the syndrome and the possible audiologist interventions for a specific case will be reported. The patient was attended at the Clinic of Speech Therapy, Faculdade de Odontologia de Bauru (FOB). She is 53 years old and presented audiological complaints. She was diagnosed with VKHS by a specialist doctor. Throughout the audiologist assessment, she presented bilateral sensorineural hearing loss, absent otoacoustic evoked emissions, 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. Therefore, before the hearing aid (HA) fitting, the audiologist should perform the hearing management, investigate if the patient takes the drug treatment and the occurrence of suggestive symptoms of the syndrome. These are some points that help in the reference to the specialist doctor and the audiologist strongly participates in what concerns the hearing.
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Humanos , Femenino , Acúfeno , Síndrome Uveomeningoencefálico/complicaciones , Síndrome Uveomeningoencefálico/diagnóstico , Pérdida Auditiva , Pérdida Auditiva Sensorineural/diagnóstico , Pérdida Auditiva Sensorineural/etiología , Vértigo , Persona de Mediana EdadRESUMEN
ABSTRACT Vogt Koyanagi Harada disease affects several parts of the body, such as eyes, meninges, ears, and skin. The progressive course of the disease can lead to blindness and deafness. The case is presented of a Hispanic woman (mixed-race) with visual alterations, headache, tinnitus, hearing loss, and posterior uveitis with serous detachments of the retina in both eyes, as well as lymphocytic meningitis. The aim of the present study is to review the literature, the diagnostic strategies, and the appropriate treatment, as well as to update the immunogenetic pathogenesis of the disease.
RESUMEN La enfermedad de Vogt Koyanagi Harada compromete múltiples órganos tales como ojos, meninges, oídos y piel. El curso progresivo de la enfermedad puede llevar a ceguera y cofosis. Se describe un caso de esta enfermedad en mujer hispana (mestiza) con alteraciones visuales, cefalalgia, tinnitus e hipoacusia a quien se le encuentra uveítis posterior con desprendimientos serosos de retina en ambos ojos y meningitis linfocitaria. El objetivo del presente estudio es, mediante una revisión de la literatura, actualizar la patogénesis inmunogenética, conocer las estrategias diagnósticas y el tratamiento apropiado.
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Humanos , Femenino , Adulto , Uveítis Posterior , Síndrome Uveomeningoencefálico , Trastornos de la Visión , Patogenesia HomeopáticaRESUMEN
Resumo Relatamos um caso atípico de uma paciente de 40 anos com apresentação completa da Síndrome de Vogt-Koyanagi-Harada (SVKH) que após 17 anos do diagnóstico inicial evoluiu com descolamento seroso de coroide. A paciente procurou atendimento com queixa de dor em olho esquerdo (OE). O exame oftalmológico revelou acuidade visual (AV) igual a de movimento de mãos, à biomicroscopia foi observada reação inflamatória granulomatosa na câmara anterior, a tonometria foi igual a 0 mmhg, e a fundoscopia indevassável pela pouca midríase e turvação de meios em OE. O descolamento seroso de coroide foi avaliado através de ultrassonografia ocular. A abordagem terapeutica intituida para paciente consistiu em prednisona 1mg/kg/dia via oral, dexametasona 1mg/mL e atropina 1% colírios. A evolução do quadro foi satisfatória, com melhora da AV para 20/40, ausência de reação inflamatória em câmara anterior, normalização da pressão intraocular e resolução do descolamento seroso de coroide em OE. Concluimos que a fase crônica da SVKH, apesar da manifestação classicamente descrita ser uveíte anterior, pode ter outras apresentações e o descolamento seroso da coroide é uma rara complicação.
Abstract We report an atypical case of a 40-year-old woman with complete presentation of Vogt-Koyanagi-Harada Syndrome (VKH) who presented with unilateral serous choroidal detachment 17 years after the diagnosis. The patient complained of pain in the left eye, the ophthalmologic examination revealed visual acuity (VA) equal to hand motion; biomicroscopy revealed a granulomatous inflammatory reaction in the anterior chamber, tonometry was equal to 0 mmhg, and the fundoscopy was impracticable. Serous choroidal detachment was assessed by ocular ultrasonography. The therapeutic approach proposed for the patient consisted of prednisone 1mg / kg / day orally, dexamethasone 1mg / mL and atropine 1% eye drops. The evolution of the condition was satisfactory, with VA improvement to 20/40, absence of inflammatory reaction in anterior chamber, normalization of intraocular pressure and resolution of serous choroidal detachment in OS. We conclude that the chronic phase of VKH, although classically described as anterior uveitis, may have other presentations and the serous choroid detachment is a rare complication.
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Humanos , Femenino , Adulto , Desprendimiento de Retina/complicaciones , Enfermedades de la Coroides/complicaciones , Panuveítis/diagnóstico , Síndrome Uveomeningoencefálico/diagnóstico , Síndrome Uveomeningoencefálico/etiologíaRESUMEN
Objective To compare the differences of optic nerve head (ONH) parameters and the thickness of circumpapillary retinal nerve fiber layer (CP-RNFL) between acute Vogt-Koyanagi-Harada syndrome (VKH) and acute central serous chorioretinopathy (CSC) patients.Methods Retrospective clinical case control analysis.This study included 38 eyes of 20 acute VKH patients (VKH group) and 37 eyes of 37 acute CSC patients (CSC group).Seventy five eyes of 57 normal healthy subjects,matching patients with age and gender,were collected as control group.The disc RPE angle,the thickness of average CP-RNFL,the nasal,superior,temporal and inferior quadrant CP-RNFL thickness,and ONH parameters including optic disc area,cup area,rim area,C/D area ratio,linear CD ratio (CDR),vertical CDR were measured by 3D-OCT.Analysis of variance was performed for comparison among three groups.Minimum significant difference t test was performed for comparison between two groups.Results The differences of ONH parameters between VKH group and CSC group:29 eyes of VKH group appeared retinal detachment next to disc,only 12 eyes appeared in CSC group.Twenty one eyes of VKH group appeared optic disc hyperemia while none in CSC group.The three groups' disc RPE angles were (138.62± 11.96)°,(154.09±5.85)° and (153.41 ±5.77)°.VKH group were significantly smaller than CSC group (t=-2.05,P=0.00) and control group (t=-1.68,P=0.00),while there was no significant difference between CSC group and control group (t=-1.88,P=0.72).The optic cup area and rim area were significantly bigger in VKH group than in CSC group (t=4.61,2.71;P=0.00,0.01),and the thickness of mean CP-RNFL,all quadrants of CP-RNFL were significantly thicker in VKH group than in CSC group (t=6.25,4.40,3.53,5.48,2.69;P=0.00,0.00,0.00,0.00,0.01).Conclusion Compared with the acute CSC,VKH patients are likely to appear retinal detachment next to disc,their disc RPE angles are smaller,their optic cup area and rim area are bigger,and their CP-RNFL thickness are thicker.
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Resumo Objetivo: Avaliar em qual fase da síndrome de Vogt-Koyanagi-Harada (SVKH) os pacientes recebem o primeiro atendimento em serviço especializado. Métodos: Foram analisados prontuários de 14 pacientes atendidos no Setor de Uveítes do Hospital Universitário Clementino Fraga Filho da UFRJ no período de janeiro de 2014 a março de 2017. Nesta análise, foram observados o sexo, a idade, a fase da doença e a acuidade visual destes pacientes com ao menos doença provável da SVKH. Resultados: Observamos que 35,4% dos pacientes apresentavam a doença ainda na fase uveítica e que 78,5% destes pacientes apresentava acuidade visual igual ou pior que 0,05. Destes pacientes, 78,5% eram do sexo feminino e 21,5% do sexo masculino e a mediana de idades foi de 34 anos. Conclusão: Os pacientes analisados obtiveram dificuldade em ter acesso precoce a um setor especializado, afetando assim, diretamente o tratamento e prognóstico visual.
Abstract Objective: To evaluate in which phase of Vogt-Koyanagi-Harada (VKH) syndrome the patients receive the first attendance in specialized service. Methods: A retrospective study was conduted to evaluate medical records of 14 patients with VKH in the Clementino Fraga Filho University Hospital of the Federal University of Rio de Janeiro from January 2014 to March 2017. In this analysis, gender, age, stage of disease and visual acuity of these patients with at least probable VKH were recorded. Results: Of these patients, 78.5% were female and 21.5% male and the median age was 34 years. We observed that 35.4% of the patients had the disease still in the uveitic phase and that 78.5% of these patients had visual acuity equal to or worse than 0.05. Conclusion: There is a delay in the admission of these patients to a specialized sector, thus affecting directly the treatment and visual prognosis.
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Humanos , Masculino , Femenino , Niño , Adolescente , Adulto , Persona de Mediana Edad , Síndrome Uveomeningoencefálico/diagnóstico , Síndrome Uveomeningoencefálico/prevención & control , Pronóstico , Agudeza Visual , Panuveítis , Registros Médicos , Enfermedad Aguda , Enfermedad Crónica , Estudios Transversales , Estudios Retrospectivos , Progresión de la Enfermedad , Diagnóstico Precoz , Diagnóstico Tardío , Intervención Médica Temprana , Estudio Observacional , Accesibilidad a los Servicios de SaludRESUMEN
Objective: To measure vascular density in retinal and choroidal capillary layers by optical coherence tomography angiography (OCTA) and to explore their potential clinical values in Vogt-Koyanagi-Harada disease (VKH). Methods: This is a cross-sectional case-control study. Twenty-one acute VKH cases presented to Peking Union Medical College Hospital between April 2015 and July 2015 and 49 healthy controls were enrolled in this study. OCTA was performed with a split-spectrum amplitude-decorrelation angiography-optical coherence tomography (SSADA-OCT) system borrowed from Optovue Inc. Vascular densities of the capillary layers were analyzed by the built-in software. Bilateral independent sample t-test was used to compare retinal and choroidal vascular density of eyes in acute phase VKH with healthy controls. Spearman correlation test was used to analyze the correlation between the vascular density of retina and choroid in acute phase VKH and LogMAR best corrected visual acuity (BCVA). Results: Mean vascular density of superficial capillary plexus (SCP), deep capillary plexus (DCP) and choriocapillaris (CC) in eyes of the acute VKH group were statistically lower than those of healthy control group (51.56%±2.88%, 56.51%±2.31%, 58.14%±2.10% vs. 53.55%±2.51%, 58.14%±2.10%, 58.14%±2.10%, t=-3.890, 3.910,-6.554, all 3 P values were<0.01). In VKH patients, eyes with retinal detachment (RD) had statistically lower mean vascular density in the CC layer than those without (NRD) (63.46%±2.19% vs. 65.85%±1.79%, t=3.890, P<0.01), while no statistically significant differences were found in vascular densities of SCP and DCP layers between RD and NRD eyes. Spearman's correlation test revealed a negative correlation between vascular density of the CC layer and logMAR BCVA(r=-0.437, P<0.01), while no statistically significant associations were found between logMAR BCVA and vascular densities of SCP and DCP. Conclusions: OCTA can be used for quantitative detection of vascular density of retina and choroidal capillary in acute VKH. There is circulatory disturbance in SCP, DCP and CC of acute VKH. Vascular density of CC is associated with visual acuity and retinal detachment. (Chin J Ophthalmol, 2017, 53: 735-739).
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Angiografía con Fluoresceína , Tomografía de Coherencia Óptica , Síndrome Uveomeningoencefálico , Enfermedad Aguda , Estudios de Casos y Controles , Estudios Transversales , Humanos , Vasos Retinianos , Estudios Retrospectivos , Síndrome Uveomeningoencefálico/diagnóstico por imagenRESUMEN
Objective: To conclude the characteristics of fundus appearance and spectral domain optical coherence tomography(SD-OCT) findings of patients with acute Vogt-Koyanagi-Harada (VKH) disease. Methods: The clinical data of 17 patients (34 eyes) diagnosed of acute VKH in Peking Union Medical College Hospital from Jan. 2012 to Dec. 2014 were studied retrospectively.Examinations included visual acuity, slit lamp, fundus, color fundus pictures, FFA and SD-OCT. Results: Eight men and 9 women were enrolled with mean age of (40.5±11.6) years old ranging from 26.0 to 62.0 years old. Vision acuity of their first consultations were as follows: 14 eyes (41.2%) were below 0.01-0.09, 17 eyes(50%) were among 0.1-0.3, 3 eyes (8.8%) were among 0.4-0.7. All patients were divided into 3 groups according to their fundus appearance: 14 eyes (41.2%) were optic disc swelling-type, 10 eyes (29.4%) were retinal detachment type and 10 eyes(29.4%)were mixed type. Subretinal fluid and serous retinal detachment appears in SD-OCT of all 34 eyes, with highly reflective signals in detached area. Other common characters were also noticeable, suh as RPE folds(19 eyes, 55.9%), subretinal septa (16 eyes, 47.1%) and internal limiting membrane(ILM) fluctuation (8 eyes, 23.5%). In addition, SD-OCT features were in accordance with multilobular dye pooling at late period of FFA. Conclusion: SD-OCT of acute VKH presents some typical features: subretinal fluid and serous retinal detachment, RPE folds, ILM fluctuation, and subretinal septa.(Chin J Ophthalmol, 2017, 53: 436-439).
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Fondo de Ojo , Tomografía de Coherencia Óptica , Síndrome Uveomeningoencefálico/diagnóstico por imagen , Enfermedad Aguda , Adulto , Femenino , Angiografía con Fluoresceína , Humanos , Masculino , Persona de Mediana Edad , Papiledema/diagnóstico por imagen , Retina/diagnóstico por imagen , Desprendimiento de Retina/diagnóstico por imagen , Estudios Retrospectivos , Microscopía con Lámpara de Hendidura , Agudeza VisualRESUMEN
Vogt-Koyanagi-Harada disease(VKH) is a bilateral, granulomatous panuveitis associated with central nervous system, auditory, and integumentary manifestations. Clinically, VKH usually responds well to early aggressive glucocorticosteroid treatment and may be cured without any clinically significant sequelae. Some patients, however, may enter the chronic recurrent phase, which may result in marked loss of vision due to complications such as complicated cataract, secondary glaucoma and maculopathy. Recurrent VKH is mainly characterized by anterior uveitis associated with thickening of the choroid. Initial poor visual acuity, severe anterior chamber reaction, choroidal folds,rapid tapering of systemic corticosteroids or inadequate duration of treatment, and development of extraocular manifestations may be risk factors of disease recurrence. Prolonged glucocorticosteroid treatment has been suggested as effective strategy for recurrence of VKH. The positive effects of other immunosuppressive agents and biologic agents on treatment of chronic recurrent and refractory VKH have been gradually recognized by the uveitis community. (Chin J Ophthalmol, 2017, 53: 317-320).