High rate of clinical recurrence in patients with Vogt-Koyanagi-Harada disease treated with early high-dose corticosteroids.

PURPOSE: To analyse the rate of clinical recurrences in Brazilian patients with Vogt-Koyanagi-Harada (VKH) disease after early high-dose corticosteroid treatment. METHODS: Retrospective study including patients treated with early high-dose corticosteroids (prednisone, 1-1.5 mg/kg/day, or 3-day 1 g methylprednisolone pulsetherapy) within 1 month from disease onset followed by slow taper (at least 6 months). Patients with a minimum 12-month follow-up were subdivided based on the presence of disease recurrence or persistence after 6 months from initial presentation into: acute-resolved (AR, no recurrences), chronic-recurrent (CR), and chronic-recurrent with subretinal fibrosis (SRF). Recurrences were defined as the presence of clinical and/or fluorescein angiography findings. RESULTS: Twenty-nine patients (58 eyes) with a median follow-up of 65 months were included. Six (21 %), 11 (38 %) and 12 (41 %) patients were allocated to AR, CR, and SRF groups respectively. Though having received treatment within 1 month of onset, median time to initial treatment differed among groups (11, 15, and 25 days, in AR, CR, and SRF groups respectively). Intensity of immunosuppression, cataract development, and longer time to achieve logMAR visual acuity ≤0.8 differed significantly among the groups, being more severe in SRF group. HLA-DRB1*0405 allele followed the same trend, though not reaching significance (0.5 in AR group, 0.6 in CR, and 0.8 in SRF). CONCLUSION: VKH disease in Brazilian patients evolved to chronic-recurrent disease in 79 % of cases; 38 % developed subretinal fibrosis, in spite of similar initial treatment regimens. Time to initiate treatment influenced outcomes.

Indocyanine green angiography findings in patients with long-standing Vogt-Koyanagi-Harada disease: a cross-sectional study.

BACKGROUND: To investigate indocyanine green angiography (ICGA) findings in patients with long-standing Vogt-Koyanagi-Harada (VKH) disease and their correlation with disease activity on clinical examination as well as with systemic corticosteroid therapy. METHODS: Twenty-eight patients (51 eyes) with long-standing (≥6 months from disease onset) VKH disease whose treatment was tapered based only in clinical features were prospectively included at a single center in Brazil. All patients underwent standardized clinical evaluation, which included fundus photography, fluorescein angiography and ICGA. Clinical disease activity was determined based in the Standardization in Uveitis Nomenclature Working Group. Fisher exact test and logistic regression models were used for statistical analysis. RESULTS: Disease-related choroidal inflammation on ICGA was observed in 72.5% (31 of 51 eyes). Angiographic findings suggestive of (choroidal and/or retinal) disease activity were not observed on FA. Clinically active disease based on clinical evaluation was observed in 41.2% (21 of 51 eyes). In these 21 eyes, disease-related choroidal inflammation on ICGA was observed in 76.2% (16 of 21 eyes); in the remaining eyes (without clinical active disease) disease-related choroidal inflammation on ICGA was observed in 70.0% (21 of 30 eyes). In respect to systemic corticosteroid therapy, 10 patients (18 of 51 eyes) were under treatment with prednisone. In these 10 (18 of 51 eyes) patients, disease-related choroidal inflammation on ICGA was observed in 83.3% (15 of 18 eyes); in the remaining patients (33 of 51 eyes) disease-related choroidal inflammation on ICGA was observed in 66.7% (22 of 33 eyes). CONCLUSION: ICGA findings suggestive of disease-related choroidal inflammation were observed in a considerable proportion of patients with long-standing VKH disease, independent of the inflammatory status of the disease on clinical examination or current use of systemic corticosteroid. Therefore, the current study reinforces the crucial role of ICGA to assist the management and treatment of patients with long-standing VKH disease.

Outcomes of Intravitreal Bevacizumab in Choroidal Neovascularization in Vogt-Koyanagi-Harada Disease- A Prospective Study.

Purpose: To assess the effectiveness of intravitreal (IV) bevacizumab and the need for systemic immunosuppressive therapy (IMT) in choroidal neovascularization (CNV) in Vogt-Koyanagi-Harada disease (VKHD).Methods: CNV treatment consisted of three monthly IV bevacizumab injections as a loading dose; if intra/subretinal fluid (IRF) persisted, further injections were proceeded besides increment in systemic IMT. Outcome analyses at 3, 6, and 12 months were visual acuity, central foveal thickness, macular volume, IRF, and addition of IMT.Results: Seven eyes of six patients were included. Five patients (five eyes) completed a 12-month follow-up and received 12 IV bevacizumab injections. At the 12-month follow-up, visual acuity improved in four out of five eyes (p = .0568); all eyes had decreased macular volume (p = .0431) but they still had persistent IRF; and all cases needed IMT introduction/increment.Conclusion: Intravitreal bevacizumab in association with systemic IMT was effective for CNV in VKHD. Active CNV may indicate disease of inadequate clinical control.

Prevalence of glaucoma in a South brazilian population: Projeto Glaucoma.

PURPOSE: To assess the prevalence of glaucoma in a South Brazilian population. METHODS: Subjects older than 40 years underwent a screening examination that included a medical interview, slit lamp examination, tonometry, and fundoscopy. Those with suspected glaucoma (based on optic disc appearance and/or intraocular pressure) underwent a comprehensive ophthalmic evaluation during the definitive examination. Glaucoma was diagnosed based on the International Society of Geographical and Epidemiologic Ophthalmology classification. RESULTS: A total of 1636 subjects were examined (76.5% participation rate); 71% of the study population self-reported their race as white and 24% as nonwhite (most black and mixed-black/white). Glaucoma was found in 56 subjects (crude prevalence of all glaucoma: 3.4%; 95% CI, 2.5-4.3), primary open-angle glaucoma (POAG) was found in 40 (2.4%; 95% CI, 1.7-3.2), and primary angle-closure glaucoma (PACG) in 12 (0.7%; 95% CI, 0.3-1.1). Six (12%) subjects with primary glaucoma had a previous diagnosis of the disease. Nonwhite persons had a higher prevalence rate of POAG than did white participants, although this difference was not significant (3.8% vs. 2.1%, respectively, P = 0.11). Unilateral blindness due to primary glaucoma was observed in seven subjects (five POAG/2 PACG), and nonwhites had a higher rate of unilateral blindness than did whites (five versus two cases, respectively, P = 0.014). CONCLUSIONS: Compared to incidence in Hispanic and European populations, PACG was more common among South Brazilians, whereas the POAG rates were similar. The rate of undiagnosed glaucoma was almost 90%. The higher POAG prevalence in the population self-reported as nonwhite may affect the estimation of glaucoma in Brazil, as more than 40% of the population self-report their race as nonwhite.

Choroidal bulging in patients with Vogt-Koyanagi-Harada disease in the non-acute uveitic stage.

BACKGROUND: Detection of choroidal inflammation in Vogt-Koyanagi-Harada (VKH) disease is still a challenge. Progression to sunset glow fundus has been observed despite apparent good clinical control of inflammation. Indocyanine green angiography (ICGA) permits choroid inflammation detection, though it is invasive, time consuming, and costly. The purpose of the present study is to report a sign indicative of probable inflammation on enhanced depth imaging spectral-domain optical coherence tomography (EDI-OCT): a localized increase in choroidal thickness with bulging of the outer retina ('choroidal bulging') in patients with VKH disease in the non-acute uveitic stage. FINDINGS: This is a retrospective observational study. The choroidal bulging was a particular finding observed in four eyes of three patients with VKH disease in the non-acute uveitic stage (median disease duration 55.3 ± 40.3 months, range 10 to 108). This study is part of an ongoing longitudinal study in patients with VKH disease carried out in the Uveitis Service, Hospital das Clínicas, Faculdade de Medicina, Universidade de São Paulo, São Paulo, Brazil. In all eyes, the choroidal bulging was identified in the presence of anterior chamber cells and/or on fundus angiographic (fluorescein and indocyanine green) findings, indicative of disease activity. Changes in the thickness of the choroidal bulging accompanied the variation in the clinical and angiographic signs of inflammation. CONCLUSION: The choroidal bulging is a particular finding detected on EDI-OCT that may indicate ongoing inflammation in the posterior segment of the eye. This EDI-OCT feature may assist in the treatment-monitoring of patients with Vogt-Koyanagi-Harada disease in the non-acute uveitic stage.

Apresentações ultrabiomicroscópicas na Síndrome de -ris Plateau / Ultrabiomicroscopy in Plateau Iris Syndrome

Objetivo: Descrever nove casos de Síndrome de -ris Plateau com suas respectivas apresentações ultrabiomicroscópicas. Local: Ambulatório de Oftalmologia do Departamento de Oftalmologia do Hospital das Clínicas da UFPR. Métodos: Procedeu-se a descrição de nove pacientes com diagnóstico de Síndrome de -ris Plateau e suas respectivas histórias clínicas, resultados de exames oftalmológicos, UBM e tratamento adotado. Resultados: Todos os pacientes relatados neste artigo apresentavam quadro clínico, gonioscopia, evolução e resposta aos tratamentos compatíveis com a Síndrome de -ris Plateau. Três primeiros casos apresentavam quadro clínico clássico com anteriorização do corpo ciliar. Os outros seis casos apresentavam profundidade de câmara anterior normal, íris em plateau e ângulo estreito ou fechado na gonioscopia, mas com sulco ciliar presente ao exame de UBM. Conclusão: A UBM é um exame complementar importante para avaliação da Síndrome de -ris Plateau. A configuração de íris plateau foi observada em todos os nove casos mas a anteriorização do corpo ciliar em apenas três casos.