ABSTRACT
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
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
Objetivo: describir cuatro casos de Vogt Koyanagi Harada (VKH), con uveítis en fase crónica de recurrencia y la respuesta a fármacos anti TNF alfa. Diseño: reporte de casos. Métodos: se realizó estudio descriptivo tipo reporte de caso, mediante recolección y análisis de historias clínicas de pacientes adultos de un centro de referencia de enfermedades autoinmunes, que cumplieran criterios diagnósticos de VKH y que estuvieran en tratamiento con terapia biológica para dicha patología. Resultados: en el presente artículo informamos una serie de cuatro casos de VKH, de los cuales todas fueron mujeres entre los 19 y 57 años, en fase crónica y de recurrencia de la enfermedad. En todos los casos existió refractariedad al uso de esteroides a dosis altas y no respuesta a inmunosupresores como ciclosporina y metotrexate, requiriéndose instauración de terapia biológica anti TNF alfa con control de su patología. Conclusiones: En el VKH en fases tardías la respuesta a altas dosis de esteroides y a inmunosupresores convencionales puede ser fallida, ante lo cual el uso de terapia biológica con fármacos anti TNF alfa y anti CD20 es una alternativa factible, requiriéndose aún mayores estudios en este campo que permitan una prescripción eficaz y segura.
Objective: to describe four cases of Vogt Koyanagi Harada (VKH) disease, in chronic and recurrence phase that were treated with anti-TNF biological therapy and their response to this therapy. Design: cases reports. Methods: We performed a descriptive, case report with clinical chart review of adult patients in a center of autoimmune diseases who met diagnostic criteria for VKH and treated with biological therapy. Results: We report four cases of VKH treated in our center of autoimmune diseases, which were all women between 19 and 57 years, in chronic and recurrence phase of disease. In all cases there was refractory to steroid use at high doses and no response to immunosuppressive treatment as cyclosporine and methotrexate, requiring introduction of anti-TNF biological therapy to control their disease. Conclusions: In chronic phases of VKH the response to high doses of steroids and conventional immunosuppressive therapy may be failed, therefore the use of biological therapy with anti-CD20 and anti-TNF alpha is a viable alternative, still requiring further study in this fi eld to enable an eff ective and safe prescription.
Subject(s)
Uveomeningoencephalitic Syndrome/epidemiology , Uveitis/drug therapy , Retinal Detachment/therapyABSTRACT
El síndrome de Vogt Koyanagi Harada es una uveitis difusa, bilateral, aguda, granulomatosa, asociada a manifestaciones dermatológicas, auditivas y del sistema nervioso central. Es la panuveitis bilateral más frecuentemente encontrada en la población mestiza e indígena mexicana. Una respuesta autoinmune, regulada por factores genéticos y aparentemente dirigada contra los melanocitos parece ser la causa del proceso inflamatorio. El diagnóstico básicamente es clínico, aunque la florangiografía de retina y el estudio ecográfico pueden ayudar a sustentar el diagnóstico básicamente es clínico, aunque la florangiografía de retina y el estudio ecográfico pueden ayudar a sustentar el diagnóstico en algunos casos. En el presente artículo revisamos los conocimientos que en relación a fisiopatogenia, diagnóstico, pronóstico, tratamiento médico y quirúrgico se tienen actualmente
Subject(s)
Humans , Eye Manifestations , Uveomeningoencephalitic Syndrome/complications , Uveomeningoencephalitic Syndrome/epidemiology , Uveomeningoencephalitic Syndrome/physiopathology , Uveomeningoencephalitic Syndrome/drug therapyABSTRACT
Se informan tres pacientes con el sindrome de Vogt-Koyanagi-Harada, conocido tambien como uveomeningoencefalitis, entidad poco conocida, de causa no claramente determinada, caracterizada por un proceso inflamatorio que compremete uvea, retina, meninges y piel.