ABSTRACT
Introducción: El síndrome de Vogt-Koyanagi-Harada pertenece a los síndromes uveomeníngeos y es una de las principales enfermedades que amenazan la visión. Se caracteriza por una uveítis bilateral que se asocia frecuentemente con manifestaciones neurológicas, auditivas y tegumentarias. Recientemente un fenotipo particular de engrosamiento macular se ha descrito como desprendimiento bacilar asociado a esta enfermedad. Objetivo: Describir el desprendimiento bacilar de la retina mediante la tomografía de coherencia óptica de dominio espectral en un caso de Vogt Koyanagi Harada en fase uveítica. Presentación de caso: Paciente femenina de 46 años de edad atendida en el Instituto Cubano de Oftalmología "Ramón Pando Ferrer" con disminución de agudeza visual en ambos ojos y panuveitis bilateral. Fue diagnosticada con síndrome de Vogt-Koyanagi-Harada en su fase uveítica aguda con presencia de manifestaciones neurológicas y desprendimiento de la capa bacilar de la retina identificado mediante la tomografía de coherencia óptica de dominio espectral. Conclusiones: El síndrome de Vogt-Koyanagi-Harada aguda y el desprendimiento bacilar de la capa de la retina pueden diferenciarse de otros signos mediante la tomografía de coherencia óptica de dominio espectral. Su desaparición pudiera tener una relación con la mejoría de la agudeza visual(AU)
Introduction: Vogt-Koyanagi-Harada syndrome belongs to the uveomeningeal syndromes and is one of the major vision-threatening diseases. It is characterized by bilateral uveitis, which is frequently associated with neurological, auditory and integumentary manifestations. Recently a particular phenotype of macular thickening has been described as bacillary detachment associated with this disease. Objective: To describe bacillary retinal detachment using spectral domain optical coherence tomography in a case of Vogt Koyanagi Harada in uveitic phase. Case presentation: 46-year-old female patient attended at the Cuban Institute of Ophthalmology "Ramón Pando Ferrer" with decreased visual acuity in both eyes and bilateral panuveitis. She was diagnosed with Vogt-Koyanagi-Harada syndrome in its acute uveitic phase with presence of neurological manifestations and detachment of the bacillary layer of the retina identified by spectral domain optical coherence tomography. Conclusions: Acute Vogt-Koyanagi-Harada syndrome and bacillary retinal layer detachment can be differentiated from other signs using spectral-domain optical coherence tomography. Their disappearance could be related to the improvement of visual acuity(AU)
Subject(s)
Humans , Female , Middle Aged , Uveomeningoencephalitic SyndromeABSTRACT
Vogt-Koyanagi-Harada disease (VKH) is an autoimmune multisystemic syndrome that includes bilateral intraocular inflammation, associated with exudative retinal detachments, and systemic manifestations in the auditory, integumentary, and central nervous systems. The frequency of VKH disease in the world is variable, but in Santiago, Chile, it causes approximately 17% of non-infectious uveitis, an incidence 2 to 3-fold greater than in the USA or European countries. The evidence shows that the pathogenesis of VKH would be caused by cell-mediated autoimmunity directed against melanocytes present in the uveal tissue. CD4+ T lymphocytes (especially hyperactivity of Th17 and Th1 cells), B lymphocytes, cytokines (e.g., TGF-ß, IL-2, IL-6, IL-23 and INF-γ) and chemokines appear to play an important role in the development of VKH. Several lines of evidence support that the pathogenesis of uveitis observed in VKH involves an altered pattern of micro-ribonucleic acids (miRNA) expression, driving the loss of immunological tolerance. In this review, we discuss the evidence related to regulation and altered expression of miRNA associated with Vogt-Koyanagi-Harada and other autoimmune diseases. (AU)
Subject(s)
Humans , Male , Female , Adult , Middle Aged , Uveomeningoencephalitic Syndrome/physiopathology , MicroRNAs/genetics , Autoimmune Diseases/physiopathology , Uveomeningoencephalitic Syndrome/genetics , Uveomeningoencephalitic Syndrome/epidemiologyABSTRACT
Summary The Human T-lymphotropic virus type 1 (HTLV-1), a retrovirus with oncogenic properties, affects around ten to twenty million people worldwide. The most common disorders associated with HTLV-1 infection are T-cell leukemia/lymphoma (ALT) and HTLV-1-associated myelopathy/tropical spastic paraparesis (HAM/TSP). Studies have reported other clinical manifestations in HTLV-1 seropositive patients, including inflammatory disorders, co-infections with opportunistic agents, and pulmonary diseases. Objective: Here, we aim to describe a cohort of juvenile patients with confirmed HTLV-1 infection that showed clinical manifestations other than neurological symptoms. Methodology and patients: Retrospective analysis of clinical data describing background and clinical findings of 12 juvenile patients with confirmed HTLV-1 infection, attended during January 2018 to February 2020 in a pediatric referral hospital in Cali, Colombia. Results: 11 out 12 patients were from Colombia´s Pacific coast, 10 suffered from significant nutritional deficiencies. Six exhibited dermatological findings, 3 compatible with infective dermatitis. None of the cases exhibited clinical or laboratory findings suggesting ALT or HAM/TPS. Eight patients had structural lung disease assessed by chest Computed Tomography (CT) scans; 4 of them tested positive for galactomannan antigen in bronchoalveolar fluid suggesting pulmonary aspergillosis, and 2 others exhibited a positive PCR testing for tuberculosis. Three patients were diagnosed with autoimmune disorders; 1 patient with Crohn´s Diseases, 1 case of autoimmune thrombocytopenic purpura, and a patient with Vogt-Koyanagi-Harada syndrome (non-granulomatous uveitis). Conclusions: There is a broad range of clinical manifestations in pediatric HTLV-1 patients, and the clinician should consider structural pulmonary disease, opportunistic co-infections and autoimmune disorders in the diagnostic algorithm.
Resumen El Virus Linfotrópico de células T humanas tipo 1 (HTLV-1), retrovirus con propiedades oncogénicas, afecta alrededor de 10-20 millones de personas mundialmente. Las manifestaciónes más comúnmente asociadas a HTLV-1 incluyen leucemia/linfoma de células T (ALT) y mielopatía asociada a HTLV-1/ paraparesia espástica tropical (HAM/TSP). Estudios han reportado otras manifestaciones clínicas en pacientes positivos para HTLV-1, incluyendo enfermedades inflamatorias, coinfecciones con gérmenes oportunistas y enfermedad pulmonar. Objetivo: es describir clínicamente una cohorte de pacientes pediátricos con infección por HTLV-1 confirmada que presentan manifestaciones clínicas diferentes a síntomas neurológicos. Metogolodía y pacientes: Análisis retrospectivo de historia clínica describiendo procedencia y hallazgos clínicos en 12 pacientes con infección por HTLV-1 confirmada, atendidos durante el periodo de Enero de 2018 a Febrero de 2020 en un hospital pediátrico de referencia en Cali, Colombia. Resultados: Once de 12 pacientes procedían de la costa Pacífica Colombiana, 10 con deficiencias nutricionales significativas. Seis mostraron compromiso dermatológico, 3 compatibles con dermatitis infectiva. Ningún paciente presentó hallazgos clínicos o paraclínicos sugestivos de ALT o HAM/TPS. Ocho pacientes presentaron enfermedad pulmonar estructural evidenciada por TAC de tórax; 4 de ellos con antígeno galactomanan positivo en lavado broncoalveolar, sugiriendo aspergilosis pulmonar, y otros 2 resultaron con PCR positiva para tuberculosis. Tres pacientes presentaron enfermedades autoinmunes concomitantes: uno con Enfermedad de Crohn, uno con Púrpura Trombocitopénica Autoinmune, y un paciente con Síndrome de Vogt-Koyanagi-Harada. Conclusiones: Existe un amplio rango de manifestaciones clínicas en pacientes pediátricos con HTLV-1, considerando enfermedad pulmonar estructural, coinfecciones oportunistas y enfermedades autoinmunes dentro del algoritmo diagnóstico.
Subject(s)
Humans , Infant , Child, Preschool , Child , Adolescent , Viruses , Human T-lymphotropic virus 1 , Infections , Paraparesis, Tropical Spastic , Leukemia , Crohn Disease , Coasts , Concurrent Symptoms , Uveomeningoencephalitic Syndrome , Purpura, Thrombocytopenic, Idiopathic , Malnutrition , Dermatitis , Pulmonary Aspergillosis , Lung DiseasesABSTRACT
El síndrome de Vogt-Koyanagi-Harada, antes conocido como síndrome uveomeningoencefálico, es una enfermedad rara, con muy pocos casos descritos en la literatura. Es una patología autoinmune infrecuente, con un compromiso multiorgánico. La resonancia magnética es importante para identificar las lesiones de la sustancia blanca periventricular, la afectación del tallo cerebral y los pedúnculos cerebrales, así como el realce paquimeníngeo y el engrosamiento difuso de la coroides. A pesar de ser una entidad poco frecuente, es importante que el radiólogo esté familiarizado con sus características en imágenes
Vogt-Koyanagi-Harada syndrome, formerly kwon as uveomeningoencephalitic syndrome, is a rare disease, with very few cases reported in the literature. It is an uncommon autoimmune entity with multi-organ involvement. Magnetic resonance imaging is important to identify periventricular white matter lesions, brain stem and brain peduncles involvement, as well as pachymeningeal enhancement and diffuse choroidal thickening. Despite being a rare entity, it is important for the radiologist be acquainted with its imaging findings
Subject(s)
Uveomeningoencephalitic Syndrome , Magnetic Resonance Imaging , Central Nervous SystemABSTRACT
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.
Subject(s)
Humans , Female , Adult , Retinal Detachment/drug therapy , Methylprednisolone/therapeutic use , Uveomeningoencephalitic Syndrome/drug therapy , Adrenal Cortex Hormones/therapeutic use , Pulse Therapy, Drug/methods , Glucocorticoids/therapeutic useABSTRACT
OBJECTIVE@#To evaluate the value of fundus autofluorescence (FAF) imaging combined with spectral domain optical coherence tomography (SD-OCT) in diagnosis, prognostic assessment and follow-up observation of acute Vogt-KoyanagiHarada (VKH) disease.@*METHODS@#Clinical data were collected from 12 patients (23 eyes) with acute VKH disease treated in our hospital from May, 2018 to November, 2019, including detailed medical history, best corrected visual acuity (BCVA), and results of slit lamp biomicroscopy, fundus photography, SD-OCT, fundus fluorescein angiography (FFA) and FAF imaging.SDOCT and FAF imaging were repeated after a course of treatment and in follow-up examination, and the results were compared with those at the time of admission.@*RESULTS@#VKH disease involved both eyes in 11 patients (91.7%).Fundus photography showed optic disc edema in 16 eyes (69.6%), and multiple retinal neuroepithelial detachment was detected by SD-OCT in all the involved eyes (100%).IN all the eyes, FFA revealed small and dense fluorescein leakage in the early stage and fluorescein accumulation in advanced stages of VHK disease to form multiple dye pooling in the areas of serous detachment.Hyperauto fluorescence was a common finding in FAF imaging (100%), and the area involved was consistent with that of fluorescein accumulation shown by FAF imaging.Ten eyes (43.5%) showed patches of relative hypoautofluorescence in the hyperauto fl uorescence areas, and granular hyperauto fl uorescence was found in the lesions in 4 eyes (17.4%).During the remission period of VKH disease, FAF imaging showed normal finding in 8 eyes (34.8%) and reduced areas (by 55.2%) and intensity (by 46.5%) of hyperautofluorescence in 9 eyes (39.1%).In 6 eyes (26.1%), only a few hyperautofluorescent spots scattered in the macula were observed.SD-OCT demonstrated significantly reduced (by 69.5% on average) or even disappearance of subretinal fluid in the eyes.The fluorescence intensity in FAF imaging showed a significant positive correlation with the volume of subretinal fluid detected by SD-OCT (@*CONCLUSIONS@#The combination of fluorescein angiography, FAF imaging and SD-OCT can significantly improve the diagnostic accuracy of VKH disease.FAF imaging combined with SD-OCT provides an effective and noninvasive modality for evaluation of remission and monitoring the changes in VKH disease.
Subject(s)
Humans , Acute Disease , Fluorescein Angiography , Follow-Up Studies , Retinal Detachment/diagnostic imaging , Tomography, Optical Coherence , Uveomeningoencephalitic Syndrome/diagnostic imagingABSTRACT
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.
Subject(s)
Humans , Female , Middle Aged , Azathioprine/toxicity , Azathioprine/therapeutic use , Uveomeningoencephalitic Syndrome/drug therapy , Chemical and Drug Induced Liver Injury/diagnosis , Immunosuppressive Agents/toxicity , Immunosuppressive Agents/therapeutic use , Sinusitis/drug therapy , Azathioprine/adverse effects , Thorax/diagnostic imaging , Radiography , Cholestasis, Intrahepatic/diagnosis , Cholestasis, Intrahepatic/blood , Ultrasonography , Pneumonia, Bacterial/drug therapy , Chemical and Drug Induced Liver Injury/blood , Goiter, Nodular/diagnostic imaging , Immunosuppressive Agents/adverse effects , Anti-Bacterial Agents/therapeutic useABSTRACT
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.
Subject(s)
Humans , Female , Adult , Uveitis, Posterior , Uveomeningoencephalitic Syndrome , Vision Disorders , Pathogenesis, HomeopathicABSTRACT
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.
Subject(s)
Humans , Female , Tinnitus , Uveomeningoencephalitic Syndrome/complications , Uveomeningoencephalitic Syndrome/diagnosis , Hearing Loss , Hearing Loss, Sensorineural/diagnosis , Hearing Loss, Sensorineural/etiology , Vertigo , Middle AgedABSTRACT
No abstract available.
Subject(s)
Adult , Female , Humans , Adalimumab , Uveomeningoencephalitic SyndromeABSTRACT
RESUMEN La patología autoinmune de oído interno se caracteriza por presentar hipoacusia sensorioneural bilateral, asimétrica y progresiva, que responde a terapia médica, presentándose aislada o como parte de una enfermedad sistémica como la enfermedad de Vogt-Koyanagi-Harada. Se presenta el caso de una paciente que consulta por cefalea, visión borrosa y sintomatología audiológica, con diagnóstico de enfermedad de Vogt-Koyanagi-Harada. Revisamos la literatura disponible respecto a la enfermedad y sus manifestaciones otológicas.
ABSTRACT Autoimmune inner ear disease presents a bilateral, asymmetric and progressive sensorineural hearing loss, that responds to medical therapy, presenting alone or associated to a systemic disease such as Vogt-Koyanagi-Harada disease. We present the case of a patient with headache, blur vision and audiologic symptoms, diagnosed with Vogt-Koyanagi-Harada disease. We review the literature about the disease and its otologic manifestations.
Subject(s)
Humans , Female , Middle Aged , Uveomeningoencephalitic Syndrome/complications , Hearing Loss, Sensorineural/etiology , Audiometry , Uveitis , Uveomeningoencephalitic Syndrome/drug therapy , Headache , Hearing Loss, Sensorineural/diagnosisABSTRACT
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.
Subject(s)
Humans , Female , Adult , Retinal Detachment/complications , Choroid Diseases/complications , Panuveitis/diagnosis , Uveomeningoencephalitic Syndrome/diagnosis , Uveomeningoencephalitic Syndrome/etiologyABSTRACT
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.
Subject(s)
Humans , Male , Female , Child , Adolescent , Adult , Middle Aged , Uveomeningoencephalitic Syndrome/diagnosis , Uveomeningoencephalitic Syndrome/prevention & control , Prognosis , Visual Acuity , Panuveitis , Medical Records , Acute Disease , Chronic Disease , Cross-Sectional Studies , Retrospective Studies , Disease Progression , Early Diagnosis , Delayed Diagnosis , Early Medical Intervention , Observational Study , Health Services AccessibilityABSTRACT
PURPOSE: To report a case of Vogt-Koyanagi-Harada (VKH) disease with chronic recurrence, which was treated with intravitreal dexamethasone implantation. CASE SUMMARY: A 39-year-old female presented with decreased visual acuities in both eyes accompanied by headaches and tinnitus the previous week. On fundus examination and optical coherence tomography, multiple serous retinal detachments with subretinal septa were observed in both eyes, and she was diagnosed with VKH disease. After undergoing high dose intravenous steroid therapy at another hospital, she was treated with oral prednisolone and immunosuppressant drugs. However, she showed frequent recurrences, and also complained of side effects from prolonged systemic steroids. We then performed intravitreal injections of dexamethasone implants in both eyes at 3-week intervals. One month following the injections, the inflammations in the anterior chamber and vitreous opacities were improved. Until the last visit at 6 months after the injection, there was no significant recurrence of inflammation, although oral prednisolone had been tapered. CONCLUSIONS: In VKH disease, intravitreal dexamethasone implantation may be an effective treatment option if chronic recurrence occurs, despite systemic steroid and immunosuppressant therapy or sustained treatment being difficult to maintain because of side effects.
Subject(s)
Adult , Female , Humans , Anterior Chamber , Dexamethasone , Headache , Inflammation , Intravitreal Injections , Prednisolone , Recurrence , Retinal Detachment , Steroids , Tinnitus , Tomography, Optical Coherence , Uveitis , Uveomeningoencephalitic Syndrome , Visual AcuityABSTRACT
PURPOSE: To report a case of atypical Vogt–Koyanagi–Harada disease that occurred after an acute angle closure glaucoma attack. CASE SUMMARY: A 48-year-old female presented with bilateral visual disturbance accompanied by headache and ocular pain. The patient had no specific past medical or family history except taking oral contraceptives for 10 years. Despite the normalization of intraocular pressure in a local clinic, a shallow-depth anterior chamber and forward displacement of the iris–lens diaphragm remained unresolved. The depth of the anterior chamber had increased in both eyes after laser therapy but without recovery of her visual acuity. B-scans showed ciliochoroidal effusion. Anterior chamber inflammation was observed in both eyes. Optical coherence tomography showed lobulated and serous retinal detachment involving the macula of both eyes. However, fluorescence angiography findings showed no multiple hyperfluorescence, which is unusual for typical cases of Vogt–Koyanagi–Harada disease. The patient was diagnosed with atypical Vogt–Koyanagi–Harada disease and was treated with eyedrops and intravenous steroid pulse therapy, after which she was converted to oral medications with immunosuppressants. After 1 month, no serous retinal detachment was detected. After 3 months, best corrected visual acuity (logMAR) was 0.0 in both eyes, and there has been no recurrence on follow-up. CONCLUSIONS: Atypical Vogt–Koyanagi–Harada disease at presentation can mimic acute attacks of angle closure glaucoma. Therefore, if there is no improvement after treatment for angle closure glaucoma including laser iridotomy, other diseases including Vogt–Koyanagi–Harada disease must be considered and the patient should be closely monitored.
Subject(s)
Female , Humans , Middle Aged , Anterior Chamber , Contraceptives, Oral , Diaphragm , Fluorescein Angiography , Follow-Up Studies , Glaucoma, Angle-Closure , Headache , Immunosuppressive Agents , Inflammation , Intraocular Pressure , Laser Therapy , Ophthalmic Solutions , Recurrence , Retinal Detachment , Tomography, Optical Coherence , Uveomeningoencephalitic Syndrome , Visual AcuityABSTRACT
Vogt-Koyanagi-Harada (VKH) syndrome is a systemic inflammatory disease that causes chronic and bilateral granulomatous panuveitis, usually described in adults. Objectives: To describe manifestations and complications of VKH in pediatric patients. Methods: Retrospectivedescriptive study upon patients <14 years-old with VKH, attended from January 1985 to July 2010 in three different centers. Results: A total of 17 patients (34 eyes) were studied; 9 (53%) female. The mean age was 10.8 years-old. Among extraocular manifestations; neurological (71%), dermatological (29%) and auditive (24%) signs were observed. Ocular findings included optic-disc involvement (94%), anterior uveitis (79%), choroiditis (77%), serous retinal detachment (71%) and vitritis (71%). Initial visual acuity (VA) was ≤0.05 in 47% of cases and ≥0.6 in 12% of patients. 71% presented complications: glaucoma (20 eyes), sinechiae (10 eyes), maculopathy (6 eyes) cataract (5 eyes) and ptisis bulbi (1 eyes). 35% received only corticosteroids and 65% inmunosupressive drugs. After treatment, 6% had VA ≤0.05 and 59% ≥0.6. Ten patients (59%) recurred: 30% compromising posterior pole, and 50% recurred >3 times. Conclusions: VKH in children is infrequent. It presents with optic-disc involvement and complications of posterior pole. It requires a high degree of suspicion, quick evaluation and early treatment, which include inmunosupressive and extended corticosteroid therapy. Nevertheless, a high rate of recurrence is seen among this group of patients. (AU)
Subject(s)
Humans , Male , Female , Child, Preschool , Child , Adolescent , Uveomeningoencephalitic Syndrome/diagnosis , Uveomeningoencephalitic Syndrome/therapy , Uveomeningoencephalitic Syndrome/complications , Uveomeningoencephalitic Syndrome/epidemiologyABSTRACT
Vogt-Koyanagi-Harada disease is a rare multisystemic granulomatous autoimmune disorder affecting pigmented tissues such as the choroid, meninges, inner ear, and the skin. Neurologic symptoms are usually mild. Clinical manifestations include generalized muscle weakness, headache, meningismus, vertigo, decreased visual acuity, hearing loss and mental changes ranging from mild confusion to psychosis, hemiparesis, dysarthria, and aphasia. Seizures are very rare. We describe a case of ¹⁸F-fluorodeoxyglucose (F-18 FDG) positron emission tomography (PET) and software-fused PET-magnetic resonance imaging (MRI) in Vogt-Koyanagi-Harada disease with seizure.
Subject(s)
Aphasia , Choroid , Dysarthria , Ear, Inner , Headache , Hearing Loss , Magnetic Resonance Imaging , Meninges , Meningism , Muscle Weakness , Neurologic Manifestations , Paresis , Positron-Emission Tomography , Psychotic Disorders , Seizures , Skin , Uveomeningoencephalitic Syndrome , Vertigo , Visual AcuityABSTRACT
PURPOSE: To report clinical features of patients with retinal and choroidal diseases presenting with acute visual disturbance during pregnancy. METHODS: In this retrospective case series, patients who developed acute visual loss during pregnancy (including puerperium) and visited a tertiary hospital from July 2007 to June 2015, were recruited by searching electronic medical records. Patients were categorized according to the cause of visual loss. Clinical features and required diagnostic modalities were analyzed in the retinal and choroidal disease group. RESULTS: Acute visual loss occurred in 147 patients; 49 (38.9%) were classified into the retinal and choroidal group. The diagnoses included central serous chorioretinopathy (22.4%), hypertensive retinopathy with or without pre-eclampsia (22.4%), retinal tear with or without retinal detachment (18.4%), diabetic retinopathy progression (10.2%), Vogt-Koyanagi-Harada disease (4.1%), retinal artery occlusion (4.1%), multiple evanescent white dot syndrome (4.1%), and others (14.3%). Visual symptoms first appeared at gestational age 25.9 ± 10.3 weeks. The initial best-corrected visual acuity (BCVA) was 0.27 ± 0.39 logarithm of the minimum angle of resolution (logMAR); the final BCVA after delivery improved to 0.13 ± 0.35 logMAR. Serious visual deterioration (BCVA worth than 20 / 200) developed in two patients. Differential diagnoses were established with characteristic fundus and spectral-domain optical coherence tomography findings in all cases. CONCLUSIONS: In pregnant women with acute visual loss, retinal and choroidal diseases are common and could be vision threatening. Physicians should be aware of pregnancy-associated retinal and choroidal diseases and their clinical features. The differential diagnosis can be established with non-invasive techniques.
Subject(s)
Female , Humans , Pregnancy , Central Serous Chorioretinopathy , Choroid Diseases , Choroid , Diabetic Retinopathy , Diagnosis , Diagnosis, Differential , Electronic Health Records , Gestational Age , Hypertensive Retinopathy , Pre-Eclampsia , Pregnant Women , Retinal Artery Occlusion , Retinal Detachment , Retinal Diseases , Retinal Perforations , Retinaldehyde , Retrospective Studies , Tertiary Care Centers , Tomography, Optical Coherence , Uveomeningoencephalitic Syndrome , Visual AcuityABSTRACT
Ocular inflammation from various causes may have similar clinical presentation thus careful clinical evaluations are mandatory particularly when the disease appear to be resistant to treatment. This paper reports a case of ocular lymphoma which was initially treated as Vogt Koyanagi Harada disease at a different centre. A 65-year-old Sudanese man complained of gradual worsening left eye vision. Careful ocular and slit lamp examination revealed a conjunctival lesion with choroidal infiltration as well as exudative retinal detachment. Computed tomography scan (CT scan) showed left eye axial proptosis, and a homogenous enhancing mass at the posterior coat of the globe. Tissue biopsy of the conjunctival lesion revealed marginal Zone B cell (MALT type), low grade, non-Hodgkin’s lymphoma. AS the ocular signs and symptoms progressed, chemotherapy was initiated. The proptosis, exudative retinal detachment, disc hyperemia and swelling improved after chemotherapy. The post treatment CT scan showed reduction of the posterior ocular coat mass. The possibility of malignant lymphoma should be considered in patients with resistant uveitis despite administration of corticosteroid. Ocular lymphoma is an indolent tumour with good prognosis. Careful ocular examination, adequate imaging studies followed by early surgical biopsy will contribute to early diagnosis.
Subject(s)
Uveomeningoencephalitic SyndromeABSTRACT
PURPOSE: To evaluate the therapeutic effect and safety of mycophenolate mofetil (MMF) on chronic uveitis in Korean patients. METHODS: This study included 25 patients with chronic uveitis who used MMF and were followed up more than 6 months in 2 referral centers from 2010 to 2014. The medical records were analyzed retrospectively. The therapeutic effect was assessed based on control of inflammation, corticosteroid sparing effects, and discontinuation of MMF, and the safety was assessed based on side effects. Control of inflammation was defined as no active inflammation observed on at least 2 consecutive visits 28 days apart or more. RESULTS: The 25 patients consisted of 18 males and 7 females. The mean age of the patients was 47.52 years. The etiology of uveitis was as follows: Behcet's disease in 15 patients (60%), Vogt-Koyanagi-Harada disease in 4 (16%), sympathetic ophthalmia in 2 (8%), systemic lupus erythematosus in 1 (4%), and idiopathic uveitis in 3 (12%). Anatomic classification was anterior uveitis in 20% and posterior uveitis or panuveitis in 80% of patients. Complete control of inflammation was achieved in 44% and 50% of patients within 6 months and 1 year, respectively. Systemic corticosteroid dosage was reduced to 10 mg of prednisone or less while maintaining sustained control of inflammation in 36% and 45% of patients for 6 months and 1 year, respectively. MMF was discontinued in 3 patients (12%) due to side effects and in 2 patients (8%) due to lack of effectiveness. CONCLUSIONS: MMF was effective and side effects were uncommon when managing chronic uveitis in Korean patients.