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AIMS: To investigate the changes in quality-of-life (QoL) metrics at a 24-month interval in non-acute VKHD patients and their association with inflammation, treatment, and visual function. METHODS: SF-36 and VFQ-25 questionnaires were administered at two 24-month-apart moments to 22 non-acute VKHD patients followed for ≥12 months since acute disease onset. "Improvement," "unchanged," or "worsening" in questionnaires scores (difference >5-point) between M1 and M2 and their associations were sought. RESULTS: Absence of systemic treatment or optic disc hyperfluorescence was associated with improved general health (SF-36). Improvement in binocular contrast sensitivity resulted in better ocular pain score; absence of anterior uveitis relapse, stable fundus findings, no use of cyclosporine or no intravitreal injections resulted in unchanged/better dependency score; no intravitreal injections resulted in unchanged/better mental health score (VFQ-25). CONCLUSION: Stability/improvement in QoL scores was associated with controlled inflammation, better visual function, and no need for treatment. Subclinical inflammatory signs did not impact QoL scores.
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BACKGROUND: The tomographic finding, which has been called the "fingerprint sign" in en face reconstructions, seems to be the result of a variety of processes that cause distension of the outer plexiform layer (OPL) and the Henle fiber layer (HFL). The aim of this paper is to describe the appearance of concentric rings at the OPL/HFL interface visualized using en face reconstructions of cross-sectional optical coherence tomography images of patients with Vogt-Koyanagi-Harada disease. METHODS: Retrospective analysis of images of six eyes of three patients obtained by cross-sectional OCT imaging and en face reconstruction at the level of the OPL/HFL interface. RESULTS: All eyes presented with a dentate or saw-tooth pattern of the OPL/HFL interface on cross-sectional OCT with corresponding concentric rings on en face OCT reconstruction, consistent with the recently published "fingerprint sign". Initial OPL/HFL interface changes were observed between the first and fourth months after treatment and resolution of VKHD associated serous retinal detachments. These OPL/HFL interface changes have persisted for many years following the resolution of the active inflammation. CONCLUSIONS: Changes in the OPL/HFL interface can be identified following successful treatment of VKHD. These included both a dentate or saw-tooth pattern on cross-sectional imaging and concentric rings or the "fingerprint sign" on en face reconstructions. These changes persisted for many years despite disease quiescence.
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Aims: To evaluate associations between vision-related (VR-) and health-related (HR-) QoL metrics and inflammation and treatment in non-acute VKHD patients.Methods: Cross-sectional study in a tertiary center in Sao Paulo, Brazil with 22 patients with non-acute VKHD followed prospectively for ≥12 months since acute disease onset, with systematic evaluation and predefined treatment protocols. VR- and HR-QoL aspects were assessed by VFQ-25 and SF-36 questionnaires, respectively. Associations between the questionnaire's subscale item scores with inflammation and systemic medical therapies were assessed.Results: After generalized linear model analysis, worse VA, severe fundus changes, fluctuation of VA and fluctuation of anterior chamber cells impacted negatively on VR-QoL items. Higher cumulative total dose of corticosteroids and use of immunosuppressive therapy impacted negatively on both questionnaires.Conclusion: Worse VA, clinical inflammation and systemic treatment have a significant impact on VR- and HR-QoL questionnaires. Subclinical choroidal inflammation did not seem to impact QoL.
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Glucocorticoides/uso terapêutico , Imunossupressores/uso terapêutico , Inflamação/etiologia , Qualidade de Vida , Autorrelato , Síndrome Uveomeningoencefálica/tratamento farmacológico , Acuidade Visual , Adulto , Idoso , Brasil/epidemiologia , Estudos Transversais , Feminino , Seguimentos , Humanos , Incidência , Inflamação/diagnóstico , Inflamação/tratamento farmacológico , Masculino , Pessoa de Meia-Idade , Estudos Prospectivos , Inquéritos e Questionários , Síndrome Uveomeningoencefálica/complicações , Síndrome Uveomeningoencefálica/epidemiologia , Adulto JovemRESUMO
PURPOSE: To evaluate associations between functional and structural measurements in patients with non-acute VKHD. METHODS: In this cross-sectional study, 16 non-acute VKHD patients (32 eyes; 14 female) were evaluated with multifocal electroretinogram (mfERG), standardized automated perimetry (SAP) and optical coherence tomography (OCT)examinations. All included patients had a minimum 12 months of follow-up from acute onset and were participants of an ongoing prospective study since acute phase with systematic clinical imaging evaluations and electroretinogram examinations within a predefined treatment. Age- and gender-matched controls were included. Main outcomes were functional and structural abnormalities and their correlation; secondary outcome was correlation of these findings with clinical characteristics, including fundus abnormalities. RESULTS: SAP and mfERG parameters were significantly worse in patients than in controls. Fourteen eyes (43.7%) had disrupted ellipsoid zone (EZ); visual acuity (VA) was similar between eyes with intact or disrupted EZ. Eyes with intact and disrupted EZ differed significantly concerning N1 and P1 amplitudes and N1 peak time values on mfERG and mean sensitivity (MS), central sensitivity (CS), foveal threshold, visual field index, mean deviation (MD) and pattern standard deviation values on SAP. The area under the curve on receiver operating curves for P1 amplitude was 0.81 (cut-off value = 34.7 nV/deg2 ) and for MD value was 0.84 (cut-off value = -5.2 dB). Central retinal thickness (CRT) significantly correlated with N1 and P1 amplitudes and P1 peak time values on mfERG (r = 0.354, r = 0.442 and r = -0.405, respectively) and MD, MS, CS and fovea threshold (log values) on SAP (r = 0.372, r = 0.406, r = 0.431 and r = 0.414, respectively). Statistically significant associations were found with the presence of peripapillary atrophy and recurrent anterior uveitis with a worse MD value (p = 0.004 and p < 0.001, respectively). CONCLUSION: In non-acute VKHD, disrupted EZ and reduced CRT were correlated with impaired mfERG and SAP parameters, even in patients with good VA.
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Eletrorretinografia/métodos , Retina/fisiopatologia , Tomografia de Coerência Óptica/métodos , Síndrome Uveomeningoencefálica/diagnóstico , Acuidade Visual , Adulto , Estudos Transversais , Feminino , Humanos , Masculino , Estudos Prospectivos , Retina/diagnóstico por imagem , Síndrome Uveomeningoencefálica/fisiopatologiaRESUMO
Purpose: To assess health-related (HR-) and vision-related (VR-) quality of life (QoL) in patients with long-standing Vogt-Koyanagi-Harada disease (VKHD).Methods: Cross-sectional study of 49 patients with disease duration ≥12 months followed at Uveitis Service, Universidade de São Paulo, BR, for at least 12 months. HR- and VR-QoL were evaluated using SF-36 and NEI VFQ-25 questionnaires, respectively. Demographic, clinical and visual function data were compared with questionnaire scores.Results: After generalized linear models, lower mensal household income was associated with lower scores in both questionnaires while unemployment was associated with SF-36 questionnaire only. Treatment with peri-/intraocular medications and ocular surgery were associated with higher scores on SF-36 questionnaire. Worse visual acuity (VA), ocular complications and no ocular surgery were related to lower scores on NEI VFQ-25 questionnaire.Conclusions: On HR- and VR-QoL questionnaires difficulties perceived by patients with long-standing VKHD were mainly associated with socio-economic aspects, VA, local treatment and ocular complications.
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Nível de Saúde , Inquéritos Epidemiológicos/métodos , Qualidade de Vida , Autorrelato , Síndrome Uveomeningoencefálica/psicologia , Acuidade Visual , Adolescente , Adulto , Idoso , Segmento Anterior do Olho/diagnóstico por imagem , Estudos Transversais , Eletrorretinografia , Feminino , Angiofluoresceinografia , Seguimentos , Fundo de Olho , Humanos , Masculino , Pessoa de Meia-Idade , Oftalmoscopia , Estudos Retrospectivos , Índice de Gravidade de Doença , Fatores Socioeconômicos , Inquéritos e Questionários , Fatores de Tempo , Tomografia de Coerência Óptica , Síndrome Uveomeningoencefálica/diagnóstico , Adulto JovemRESUMO
PURPOSE: To prospectively evaluate the dynamic changes of the full-field electroretinogram (ff-ERG) and its association with inflammatory signs in patients with Vogt-Koyanagi-Harada disease (VKHD) followed up after acute onset. METHODS: Twelve acute VKHD patients, who were followed up for at least 24 months, were enrolled at a tertiary center from June 2011 to January 2017. Treatment consisted of intravenous methylprednisolone followed by 1 mg/kg/day of oral prednisone with a slow tapering associated with late non-steroidal immunosuppressive therapy in previously defined cases. Inflammation was systematically evaluated with clinical and posterior segment imaging (PSI) exams (fluorescein angiography, FA, indocyanine green angiography, ICGA, enhanced depth imaging optical coherence tomography, EDI-OCT). A ff-ERG was performed upon enrollment as well as at predefined intervals. Scotopic ff-ERG parameters changes between the 12th and 24th months defined the ERG-stable or ERG-worsening groups. "Flare" was defined as an appearance or worsening of inflammatory signs (after the initial 6 months following disease onset) under the predefined treatment protocol. RESULTS: ff-ERG parameters initially improved in all eyes; in the evaluation between the 12th and 24th months, ff-ERG results were stable in 17 eyes (71 %) and worsened in 7 eyes (29 %). Subnormal ff-ERG results were observed in 15 eyes (62 %) at the 24th month. On the other hand, the flare was observed in 8 eyes (33 %) as cells in the anterior chamber and in 24 eyes (100 %) as any PSI inflammatory sign. The ERG-worsening group presented thicker subfoveal choroid at the first month (p = 0.001) and fluctuations in choroidal thickness more often during follow-up when compared to the ERG-stable group (p = 0.02). CONCLUSIONS: Scotopic ff-ERG parameters worsened between the 12th and 24th months in a quarter of the patients. Subclinical inflammation detected as an increase in CT seems to be related to worsening in visual function measured with ffERG.
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Eletrorretinografia/métodos , Retina/fisiopatologia , Síndrome Uveomeningoencefálica/diagnóstico , Doença Aguda , Adolescente , Adulto , Idoso , Corioide/patologia , Progressão da Doença , Feminino , Angiofluoresceinografia/métodos , Seguimentos , Fundo de Olho , Humanos , Masculino , Pessoa de Meia-Idade , Prognóstico , Estudos Prospectivos , Retina/diagnóstico por imagem , Fatores de Tempo , Tomografia de Coerência Óptica/métodos , Síndrome Uveomeningoencefálica/fisiopatologia , Adulto JovemAssuntos
Biomarcadores , Imagem Óptica , Epitélio Pigmentado da Retina/patologia , Síndrome Uveomeningoencefálica/diagnóstico , Administração Oral , Adulto , Corantes/administração & dosagem , Combinação de Medicamentos , Feminino , Angiofluoresceinografia , Fundo de Olho , Glucocorticoides/uso terapêutico , Humanos , Masculino , Metilprednisolona/uso terapêutico , Pessoa de Meia-Idade , Prednisona/uso terapêutico , Estudos Prospectivos , Pulsoterapia , Índice de Gravidade de Doença , Tomografia de Coerência Óptica , Síndrome Uveomeningoencefálica/diagnóstico por imagem , Síndrome Uveomeningoencefálica/tratamento farmacológico , Adulto JovemRESUMO
Vogt-Koyanagi-Harada disease (VKHD) is a rare granulomatous inflammatory disease that affects pigmented structures, such as eye, inner ear, meninges, skin and hair. This disease is mainly a Th1 lymphocyte mediated aggression to melanocytes after a viral trigger in the presence of HLA-DRB1*0405 allele. The absence of ocular trauma or previous intraocular surgery sets VKHD appart from sympathetic ophthalmia, its main differential diagnosis. The disease has an acute onset of bilateral blurred vision with hyperemia preceded by flu-like symptoms. The acute uveitic stage is characterized by a diffuse choroiditis with serous retinal detachment and optic disc hyperemia and edema. Fluorescein angiography in this phase demonstrates multiple early hyperfluorescent points. After the acute uveitic stage, ocular and integumentary system pigmentary changes may appear. Ocular findings may be accompanied by lymphocytic meningitis, hearing impairment and/or tinnitus in a variable proportion of patients. Prompt diagnosis followed by early, aggressive and long-term treatment with high-dose corticosteroids is most often ensued by good visual outcomes. However, some patients may experience chronic uveal inflammation with functional eye deterioration. The current review discusses the general features of VKHD, including epidemiology, classification into categories, differential diagnosis and current therapeutic approaches.
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Doenças Autoimunes/metabolismo , Melanócitos/metabolismo , Doenças Raras/metabolismo , Síndrome Uveomeningoencefálica/metabolismo , Animais , Doenças Autoimunes/imunologia , Humanos , Melanócitos/imunologia , Doenças Raras/imunologia , Uveíte/imunologia , Uveíte/metabolismo , Síndrome Uveomeningoencefálica/imunologiaRESUMO
A dolicoectasia da artéria carótida interna (ACI) é uma condição rara que pode ser acompanhada de manifestações neuro-oftalmológicas, como perda da acuidade e alteração do campo visual decorrente da compressão do nervo óptico (NO). O objetivo é relatar um caso de paciente do sexo masculino, 67 anos, portador de glaucoma primário de ângulo aberto (GPAA) com evolução atípica, assimetria de escavação, palidez da rima do NO à esquerda, devido à neuropatia óptica compressiva à esquerda, por segmento dolicoectásico da ACI. O diagnóstico foi baseado na história clínica, aspecto do NO e exames de neuroimagem.
Dolichoectasia of the internal carotid artery (ICA) is a rare condition that may be associated with neuro-ophthalmic manifestations, such as loss of visual acuity and visual field resulting from compression of the optic nerve (ON). The aim is to report a 67-year-old male patient with primary open-angle glaucoma (POAG) with atypical evolution, asymmetry of cupping and increased pallor of the rim of the left ON, due to compressive optic neuropathy by the dolichoectatic segment. The diagnosis was based on clinical history, appearance of the ON and neuroimaging.
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Humanos , Masculino , Idoso , Transtornos da Visão/etiologia , Doenças das Artérias Carótidas/complicações , Doenças do Nervo Óptico/etiologia , Glaucoma de Ângulo Aberto/complicações , Síndromes de Compressão Nervosa/etiologia , Transtornos da Visão/diagnóstico por imagem , Imageamento por Ressonância Magnética , Doenças das Artérias Carótidas/diagnóstico por imagem , Artéria Carótida Interna/patologia , Artéria Carótida Interna/diagnóstico por imagem , Campos Visuais/fisiologia , Doenças do Nervo Óptico/diagnóstico por imagem , Angiografia por Ressonância Magnética , Pressão Intraocular/fisiologia , Síndromes de Compressão Nervosa/diagnóstico por imagemRESUMO
Hemoglobinopathies are a group of inherited disorders characterized by quantitative or qualitative malformations of hemoglobin (Hb). Some of these diseases present vaso-occlusive phenomena that are responsible for high morbidity in clinical and/or ophthalmologic terms. Diagnosis of hemoglobinopathies is performed exclusively through hemoglobin electrophoresis. From the ophthalmologic perspective, the most important representative of this group of diseases is sickle cell retinopathy, which presents a wide spectrum of fundus manifestations and may even lead to irreversible vision loss if not properly diagnosed and treated. The aim of this review is to present the classification of sickle cell retinopathy and to describe current management and future perspectives for its treatment, taking into consideration the clinical management of these patients.