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.
Autoantibodies/*blood/immunology ; Autoantigens ; Autoimmune Diseases/*immunology ; Electroretinography ; Humans ; Immunologic Factors ; Paraneoplastic Syndromes/*immunology ; Paraneoplastic Syndromes, Ocular ; Phosphopyruvate Hydratase ; Recoverin ; Republic of Korea ; Retina/*immunology ; Retinal Diseases/*immunology ; Tomography, Optical Coherence

OBJECTIVE: To evaluate the diagnostic performance of the AllergyScreen system from Mediwiss An alytic GmbH and the Pharmacia CAP system from Pharmacia Diagnostics for the detection of four inhalant allergens in the diagnosis of allergic rhinitis. METHOD: All 35 serum samples were collected from patients who were referred to the allergist for a suspected allergic rhinitis between January to March in 2007. Patients were classified as study diagnosis-positive for inhalant allergy if they both had a positive clinical examination/history and a related positive skin test for the suspected inhalant allergen. RESULT: Comparing with the reference standard, the diagnostic indexes (sensitivity, specificity, accuracy, predictive value of positive, and predictive value of negative) of the CAP system and the AllergyScreen system were 0.96 vs 0.89, 0.84 vs 0.75, 0.89 vs 0.80, 0.78 vs 0.65 and 0.93 vs 0.93 respectively. The CAP system method had higher sensitivity, specificity and accuracy than AllergyScreen system method, but there have no statistical difference between two systems (P>0.05). CONCLUSION This data support the use of ImmunoCAP system and AllergyScreen system to identify potentially significant individual allergens in the diagnosis of allergic rhinitis. The diagnostic indexes between the two systems have no statistical difference. As a simple, rapid turnaround time and low-cost system, AllergyScreen system can test multi-allergens in one time, so it can be used as a complementary with the ImmunoCAP system.
Adolescent ; Adult ; Allergens ; blood ; immunology ; Child ; Female ; Humans ; Immunoglobulin E ; blood ; immunology ; Male ; Middle Aged ; Predictive Value of Tests ; Recoverin ; Rhinitis, Allergic, Seasonal ; blood ; diagnosis ; immunology ; Serologic Tests ; methods ; Skin Tests ; Young Adult

PURPOSE: Cancer-associated retinopathy is a paraneoplastic retinal degeneration which may primarily result from auto-immune mediated apoptosis. It has been hypothesized that high titer of auto-antibodies are able to cross the blood-retinal barrier (BRB) and to enter retinal cells to activate apoptotic pathway which has been already well-established. However, it still remains to be elucidated whether auto-antibodies could cross BRB in the retina. Herein, we demonstrated that intravenously administrated anti-recoverin antibodies could not pass through BRB and not lead to retinal cell death. METHODS: Anti-recoverin antibody was intravenously injected to C57BL/6 mice, which were sacrificed 1 and 7 days to obtain eye. Vascular endothelial growth factor was intravitreally injected to induce BRB breakdown, which was confirmed by fluorescein angiography and western blotting for zonula occludens (ZO)-1, ZO-2 and occludin. To investigate the location of anti-recoverin antibody in the retina, immunofluorescein was performed. The retinal toxicity of intravenous anti-recoverin antibody was evaluated by histological examination and transferase-mediated dUTP nick-end labeling. Immunofluorescein staining for glial fibrillary acidic protein was done to address glial activation as well. RESULTS: Intravenously administrated anti-recoverin antibodies were exclusively distributed on retinal vessels which were co-localized with CD31, and led to neither increase of glial fibrillary acidic protein expression, as an indicator of retinal stress, nor apoptotic retinal cell death. Moreover, even in the condition of vascular endothelial growth factor-induced BRB breakdown, anti-recoverin antibodies could not migrate across BRB and still remained on retinal vessels without retinal cytotoxicity. CONCLUSIONS: Our results suggest that high titer of intravascular anti-recoverin antibodies could not penetrate into the retina by themselves, and BRB breakdown mediated by dysregulation of tight junction might not be sufficient to allow anti-recoverin antibodies to pass through BRB.
Animals ; Antibodies/*administration & dosage/*metabolism ; Blood-Retinal Barrier/*metabolism ; Cell Death/drug effects ; Cells, Cultured ; Female ; Injections, Intravenous ; Mice ; Mice, Inbred C57BL ; Recoverin/*immunology ; Retina/cytology/drug effects ; Retinal Vessels/metabolism