Non-Paraneoplastic Autoimmune Retinopathy: The First Case Report in Korea

Autoimmune retinopathy (AIR) is an immune-mediated retinopathy, resulting from an immunologic process caused by the aberrant recognition of retinal antigens as autoantigens. The diagnosis of AIR involves the detection of antiretinal antibodies with concurrent clinical and electrophysiological evidence of retinopathy. A 40-year-old patient presented with progressive loss of bilateral vision over several months. A fundus examination was unremarkable. Spectral domain optical coherence tomography revealed a blurred photoreceptor ellipsoid zone at the subfoveal region in both eyes with more prominent disruption in the left eye. Full-field electroretinography (ERG) showed relatively normal rod and cone responses in the right eye, and decreased photopic bwaves with minimal attenuation of a-waves in the left eye. Multifocal ERG demonstrated slightly reduced amplitude of the inner segment ring in the right eye and decreased amplitudes and delayed latencies of all modalities in the left eye. The patient was suspected to have AIR and it was supported by positive Western blots for 23-kDa protein, enolase (46-kDa), aldolase (40-kDa), 62-kDa and 78-kDa proteins and by immunohistochemical staining of human retinal bipolar and ganglion cells. Despite the immunosuppressive treatment, the destruction of the retinal photoreceptors progressed, and immunosuppressive interventions produced very little visual improvement. We report on what is, to the best of our knowledge, the very first case of serologically confirmed nonparaneoplastic AIR in Korea.

Birdshot retinochoroidopathy review / Revisão sobre retinocoroidopatia do tipo "birdshot"

Birdshot retinochoroidopathy (BSRC) is a distinct type of posterior uveitis originally described in the 1940s. Its characteristics include minimal anterior segment inflammation and diffuse posterior choroidopathy with vitritis and retinal vasculitis. The precise etiology of this disease is yet to be elucidated. However, various treatment modalities have been employed with the ultimate goal of durable remission of this vision threatening intraocular disease. The purpose of this review is not only to emphasize the importance of recognizing BSRC, but also to discuss the new discoveries, immune mediators, current and new therapies, and techniques applied to monitor and accomplish disease remission.

Retinocoroidopatia do tipo "birdshot" é um tipo de uveíte posterior originalmente descrita na década de 1940. Achados característicos incluem inflamação mínima do segmento anterior, retinocoroidopatia difusa associada à vitreíte e vasculite retiniana. A etiologia da doença ainda não foi completamente definida, entretanto várias modalidades de tratamento têm sido utilizadas com o objetivo de atingir a remissão. O objetivo desta revisão é enfatizar não só a importância do reconhecimento da doença como também discutir novas descobertas relacionadas a mediadores imunes, formas de tratamentos e como monitorar a doença.

Associação do sistema de histocompatibilidade humano com doenças oftalmológicas / Association of human histocompatibility antigens with ophthalmological disorders

Vários estudos têm procurado identificar marcadores genéticos para doenças oftalmológicas. Dentre eles, destaca-se o antígeno de histocompatibilidade humano (Human Leukocyte Antigens). Situado no braço curto do cromossomo 6, o sistema antígeno de histocompatibilidade humano é conhecido por sua capacidade de conferir susceptibilidade ou proteção a diferentes doenças. Em virtude do seu acentuado polimorfismo, o tipo e a força da associação variam a depender da enfermidade e da raça (etnia) estudadas. O surgimento de métodos moleculares para tipificação dos alelos antígeno de histocompatibilidade humano e as recentes atualizações de sua nomenclatura têm contribuído para o melhor entendimento desse sistema. O presente trabalho tem por objetivos revisar a estrutura e função do sistema antígeno de histocompatibilidade humano e relatar suas associações com uveíte anterior aguda, penfigóide cicatricial ocular, ceratocone de início na juventude e retinocoroidopatia "birdshot".

Evaluation of lymphocyte proliferation assay to purified protein derivative, enzyme linked immunosorbant assay, and tuberculin hypersensitivity in Eales' disease.

The purpose of this study was to evaluate the immunological responses against mycobacterial antigens in Eales' disease. Fifty six patients with Eales' disease and fifty age-and-sex-matched healthy volunteers with normal fundus findings taken as controls, were subjected to Mantoux test, using 2 TU/0.1 ml of purified protein derivative (PPD), lymphocyte proliferation assay to PPD, and ELISA to detect IgM and IgG antibodies against mycobacterial A-60 antigen. The results of Mantoux test and lymphocytes proliferation assay did not differ significantly in the two groups suggesting a similar cellular immune response. The number of individuals with recent exposure/reexposure to tuberculosis (IgM+) was significantly higher among patients. However the number of people with past exposure (IgM-IgG+) was significantly higher among controls. Our study indicates that there are no statistically significant differences in the humoral and cellular immune responses to mycobacterial antigens between the patients with Eales' disease and controls, except for a significantly higher IgM positivity among the patients.