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Objective: To characterize changes in the retinal microvasculature in eyes with birdshot chorioretinopathy (BCR) using OCT angiography (OCTA). Design: Retrospective, observational, single center. Subjects: Twenty-eight patients (53 eyes) with BCR and 59 age-matched controls (110 eyes). Methods: En face OCTA images of the superficial capillary plexus (SCP) and deep capillary plexus (DCP) of each eye were assessed for the presence of microvascular abnormalities and used to measure the vessel and foveal avascular zone (FAZ) areas. A longitudinal analysis was performed with a representative cohort of 23 BCR eyes (16 patients) at baseline and at a 2-year time point. Main Outcome Measures: Whole-image vessel density (VD, %), extrafoveal avascular zone (extra-FAZ) VD (%), and FAZ area (%) were calculated and compared between control and BCR eyes. The frequency of microvascular abnormalities in BCR eyes was recorded. Results: In the SCP, increased intercapillary space and capillary loops were common features present on OCTA images. Whole-image and extra-FAZ VD were lower in the BCR group compared with controls (P < 0.0001 [SCP and DCP]). Foveal avascular zone area was enlarged in BCR eyes (P = 0.0008 [DCP]). Worsening best-corrected visual acuity was associated with a decrease in whole-image and extra-FAZ VD in the SCP (P < 0.0001 for both) and the DCP (P < 0.005 for both). Multivariable analysis, with vessel analysis parameters as outcomes, demonstrated that increasing age, increasing disease duration, lower central subfield thickness, and treatment-naive eyes (compared with those on only biologics) were associated with a significant decrease in both DCP whole-image and extra-FAZ VD. Increasing disease duration was associated with a significant decrease in both SCP whole-image and extra-FAZ VD. Longitudinal analysis demonstrated no significant difference in any vessel analysis parameters except for an increase in DCP FAZ area. Conclusions: Our results demonstrate a significant a decrease in VD in BCR eyes and an association on multivariable analysis with disease duration. Quantifying VD in the retinal microvasculature may be a useful biomarker for monitoring disease severity and progression in patients with BCR. Further studies with extended longitudinal follow-up are needed to characterize its utility in monitoring disease progression and treatment response. Financial Disclosures: Proprietary or commercial disclosure may be found in the Footnotes and Disclosures at the end of this article.
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Purpose: To examine an underreported and underdiagnosed phenomenon with implications for the pathophysiological mechanisms of tattoo-induced uveitis. Methods: Two cases highlighting the clinical presentation of tattoo-related uveitis were evaluated. Results: A 28-year-old man with biopsy-proven sarcoidosis and ocular manifestations presented with worsening retinal vasculitis after acquiring a red-ink tattoo. Each subsequent flare followed acquisition of a new tattoo. A 31-year-old woman without systemic sarcoidosis presented with multiple episodes of bilateral intermediate uveitis and macular edema concurrent with inflammatory granulomas to recently acquired black-ink tattoos. A skin biopsy in both patients showed cutaneous noncaseating granulomas. Conclusions: These cases add to those reported in the literature and emphasize the importance of understanding the modifiable factors of inflammatory ocular disease. Future study is necessary to understand the mechanisms of tattoo-related uveitis.
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Purpose: To report a patient with severe hemophilia A who received intravitreal antivascular endothelial growth factor (anti-VEGF) for exudative age-related macular degeneration (AMD). Methods: A case and its findings were analyzed. A systematic literature was performed. Results: A patient with severe hemophilia A received intravitreal anti-VEGF for exudative AMD, after which he developed a severe subconjunctival hemorrhage requiring hemostatic intervention. The effects of anti-VEGF in patients with preexisting blood-clotting disorders, such as classic hemophilia, have not been widely reported in the current literature. Conclusions: This case illustrates that precautions must be taken when performing ocular procedures on patients with serious hemostasis disorders.
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BACKGROUND: To report a cohort of patients with clinically and genetically diagnosed autosomal dominant neovascular inflammatory vitreoretinopathy (ADNIV) and showcase the spectrum of the disease utilizing multimodal imaging and genetic testing. Additionally, the utility of multimodal imaging in guiding treatment will also be illustrated. MATERIALS/METHODS: Five patients from a single-family pedigree in Ohio with clinical signs of ADNIV were evaluated. Medical history, family history, and complete ocular examinations were obtained during regular clinic visits. Multimodal imaging including ocular coherence tomography, fluorescein angiography, wide-field fundus photographs, and Humphrey visual field testing was obtained for all five patients. Additionally, genetic testing for the Calpain-5 (CAPN5) gene was conducted on all patients. RESULTS: All five patients were noted to have a CAPN5 c.731T > C (p.L244P) mutation on genetic testing. Using multimodal imaging to supplement the clinical examination, pathologic changes such as retinal vascular inflammation, macular edema, and tractional retinal membranes were well illustrated and monitored over time. This allowed for earlier intervention when appropriate such as with intraocular steroid or systemic anti-inflammatory treatments. CONCLUSION: Phenotypic presentation varied among patients in this series, but is consistent with the spectrum of pathologic changes previously described in patients with other CAPN5 gene mutations. Monitoring of patients with ADNIV utilizing multimodal imaging can help better assess progression of this disease and guide treatment decisions. Additionally, increased genetic testing in patients with inherited retinal diseases may reveal novel gene mutations that could serve as potential targets for future genetic treatment regimens.
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Vitreorretinopatia Proliferativa , Humanos , Vitreorretinopatia Proliferativa/diagnóstico , Vitreorretinopatia Proliferativa/genética , Vitreorretinopatia Proliferativa/patologia , Mutação , Retina/patologia , Linhagem , Angiofluoresceinografia , Tomografia de Coerência ÓpticaRESUMO
We report a case of minocycline-induced ocular ochronosis with scleral, retinal, and cutaneous manifestations. A 65-year-old male who had taken minocycline for four years to treat hidradenitis suppurativa, an inflammatory skin condition affecting the apocrine sweat glands and hair follicles, presented for evaluation of discoloration of bilateral sclera, nail beds, and gingiva. Ophthalmic evaluation revealed intact visual acuity, diffuse blue-gray hyperpigmentation of the sclera, more pronounced overlying insertions of the horizontal muscles, without any scleral thinning. Macular optical coherence tomography and fundus exam revealed a blue hue to the underlying choroid with dark deposits in the retinal pigment epithelium. Despite drug discontinuation, after six years the discoloration persisted. Management was directed towards patient tolerability.
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BACKGROUND: Regional corticosteroid therapy for noninfectious uveitis is well-established but usage patterns have not been studied extensively. This study aims to assess practice patterns of retina and uveitis specialists regarding their preferences on the use of local corticosteroid therapy. METHODS: A 13-question survey was developed regarding the practice patterns of regional corticosteroid use in specific situations and populations. The survey was distributed to both the American Uveitis Society and Macula Society. RESULTS: Responses from 87 ophthalmologists were analyzed. The two most commonly used drugs were the dexamethasone intravitreal implant (Ozurdex®) and posterior sub-tenon's triamcinolone (also known as posterior sub-Tenon's Kenalog, or PSTK). Regional corticosteroids were used more frequently as first-line treatment in more than half of posterior uveitis cases when compared to anterior uveitis (39.1-46.0% vs 10.3%, respectively). Respondents were more willing to use regional corticosteroids in more than half of unilateral uveitis cases than in bilateral cases (54.7% vs 18.6%, respectively). A majority of respondents (67.1%) stated that they would avoid using regional corticosteroids in patients under 8 years old. CONCLUSIONS: Our results demonstrate more frequent regional corticosteroid use in posterior segment uveitis, unilateral cases, and avoidance in younger pediatric patients. Overall, the variability in these responses highlights the need for guidelines regarding regional corticosteroid use.
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PURPOSE: To present two patients with treatment-naïve posterior segment uveitis with Vogt-Koyanagi-Harada-like features associated with tattoo-related inflammatory skin changes. METHODS: Retrospective report of two cases. RESULTS: Using clinical history and multimodal imaging, a diagnosis of posterior segment uveitis with Vogt-Koyanagi-Harada-like features was made in association with tattoo skin changes in both patients. CONCLUSION: Physicians should be aware that tattoo-associated posterior segment uveitis with Vogt-Koyanagi-Harada-like features can occur.
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Tatuagem , Uveíte Posterior , Uveíte , Síndrome Uveomeningoencefálica , Humanos , Estudos Retrospectivos , Tatuagem/efeitos adversos , Síndrome Uveomeningoencefálica/complicações , Síndrome Uveomeningoencefálica/diagnósticoRESUMO
PURPOSE: To report the prevalence of QuantiFERON-TB Gold (QFT-G) positivity among uveitis patients compared to general population and to evaluate the differences in clinical features of uveitis. DESIGN: Retrospective cohort study. METHODS: SETTING: Institutional. PATIENT POPULATION: 418 consecutive new uveitis patients, regardless of clinical suspicion, were tested for QFT-G. OBSERVATION PROCEDURES: Demographics, TB risk factors, clinical characteristics of uveitis were collected. MAIN OUTCOME MEASURES: The frequency of QFT-G positivity among uveitis patients and characteristic clinical features among QFT-G positive patients. RESULTS: QFT-G positivity was found in 60/418 patients with uveitis (14.4%, 95% CI: 11.18 - 18.14) higher than the general US population (5%, 95% CI: 4.2 - 5.8, p<.001). Age, gender and residence were similar between QFT-G positive and negative groups. Uveitis patients with positive QFT-G were more likely to be foreign born or have a recent travel history (OR:5.84; 95% CI: 2.83 - 12.05; p<.001). QFT-G positive patients were more likely to present with granulomatous uveitis (OR 2.90; 95%CI 1.36 - 6.21; p=.006). No significant association was found with specific clinical features such as choroiditis, retinal vasculitis, occlusive vasculitis, and serpiginoid choroiditis (p>.05 for each). Prevalence of TB-uveitis based on treatment response was 1.19%. CONCLUSIONS: Our study demonstrates significantly higher prevalence of QFT-G positivity among uveitis patients compared to average US population. Characteristic signs of TB uveitis reported in endemic countries were not seen in this cohort. Implications of higher prevalence of QFT-G positivity among uveitis patients require further investigation.
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Corioidite , Tuberculose Ocular , Uveíte , Humanos , Testes de Liberação de Interferon-gama , Estudos Retrospectivos , Teste Tuberculínico , Tuberculose Ocular/diagnóstico , Tuberculose Ocular/epidemiologia , Estados Unidos/epidemiologia , Uveíte/diagnóstico , Uveíte/epidemiologiaRESUMO
Glaucoma, a leading cause of irreversible blindness, affects more than 64 million people worldwide and is expected to grow in number due to the aging global population and enhanced methods of detection. Although topical therapies are often effective when used as prescribed, the drawbacks of current medical management methods include poor patient adherence, local and systemic side effects, and in some cases, limited therapeutic efficacy. Novel ocular drug delivery platforms promise to deliver differentiated drug formulations with targeted delivery leveraging patient-independent administration. Several platforms are in various stages of development with promising pre-clinical and clinical data. The Bimatoprost Sustained Release (SR) intracameral implant was approved in the United States in March of 2020, making it the first long-term injectable therapy available for the treatment of glaucoma. This review aims to provide an update on novel sustained release drug delivery systems that are available today as well as those that might be commercialized in coming years.
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Purpose: Aims to determine the variability of ffERG measurements in patients with clinically stable birdshot chorioretinopathy (BCR).Methods: Repeatability coefficients (RC) of ffERG amplitudes and implicit times were calculated from 11 BCR patients. Jackknife resampling estimated 95% confidence intervals of each ERG parameter's RC and the percentage change explained by variability alone was calculated.Results: Intra-visit variability in ffERG parameters was lower than inter-visit. Intravisit RCs demonstrated that for intravisit ERG testing, there was less than 30% variation in ERG amplitude for most parameters. For inter-visit ERG testing, a greater than 40% reduction in ERG amplitude may be clinically meaningful for 6 of 8 ERG parameters. Photopic single flash responses have <2 msec of test-retest variability both within and across visits.Conclusions: A 40% reduction in ERG amplitude and/or a delay of >2 msec in the photopic single flash response may be suitable criteria for meaningful change in BCR patients.
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Coriorretinopatia de Birdshot/fisiopatologia , Eletrorretinografia/normas , Retina/fisiopatologia , Adulto , Idoso , Coriorretinopatia de Birdshot/diagnóstico , Adaptação à Escuridão/fisiologia , Angiofluoresceinografia , Humanos , Masculino , Pessoa de Meia-Idade , Variações Dependentes do Observador , Estimulação Luminosa , Reprodutibilidade dos TestesRESUMO
Glaucoma secondary to ocular inflammation is potentially blinding and requires aggressive multifaceted management. The pathogenesis is related to the intraocular pressure elevation secondary to compromised aqueous humor outflow by inflammatory cells, chemical mediators released into the aqueous, and to the uveitic therapy itself (corticosteroid induced). The management of uveitic glaucoma needs a careful combination between anti-inflammatory therapy and appropriate intraocular pressure-lowering drugs to prevent worsening of condition and long-term visual loss. Although there have been novel medications that have emerged in the management of glaucoma and uveitis, around 30% of uveitic glaucoma eyes do not respond to maximal medical therapy and require surgical intervention. Studied procedures include laser therapy, filtration surgery, and glaucoma drainage devices, as well as recent surgical techniques for intraocular pressure control such as minimally invasive glaucoma surgery and angle-based procedures. We review and update the medical and surgical management of uveitic glaucoma.
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Gerenciamento Clínico , Glaucoma/terapia , Pressão Intraocular/fisiologia , Hipertensão Ocular/terapia , Uveíte/complicações , Glaucoma/etiologia , Glaucoma/fisiopatologia , Humanos , Hipertensão Ocular/complicações , Hipertensão Ocular/etiologia , Hipertensão Ocular/fisiopatologiaAssuntos
Anticorpos Antibacterianos/sangue , Borrelia burgdorferi/imunologia , Infecções Oculares Bacterianas/diagnóstico , Doença de Lyme/diagnóstico , Uveíte/diagnóstico , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Criança , Ensaio de Imunoadsorção Enzimática , Infecções Oculares Bacterianas/imunologia , Infecções Oculares Bacterianas/microbiologia , Reações Falso-Positivas , Feminino , Humanos , Doença de Lyme/imunologia , Doença de Lyme/microbiologia , Masculino , Pessoa de Meia-Idade , Valor Preditivo dos Testes , Uveíte/imunologia , Uveíte/microbiologiaRESUMO
BACKGROUND Atypical hemolytic uremic syndrome (aHUS), a rare thrombotic microangiopathy, is characterized by hemolytic anemia, thrombocytopenia, and acute renal failure. Caused by genetic mutations in the alternative complement cascade, aHUS often will culminate in end-stage renal disease and occasionally death. Renal transplantation in aHUS patients has been contraindicated in the past due to the recurrence risk, with certain immunosuppressive regimens being commonly attributed. In this study, we analyzed the association between aHUS and immunosuppressive agents so as to offer evidence for the use of certain immunosuppressive regimens in renal transplant recipients. MATERIAL AND METHODS Our study is a retrospective analysis using data from the United States Renal Data System from 2004 to 2012. A cohort of renal transplantation patients diagnosed with aHUS were identified to include in the study. The primary endpoint was the determination of aHUS incidence in renal transplant recipients due to various immunosuppressive agents. The secondary endpoints were to check the relationship between the drug type as well as the demographic variables that increase the risk for aHUS. RESULTS It was found that there was a higher usage of sirolimus (P=0.015) and corticosteroids (P=0.030) in the aHUS patients compared to patients in other diagnoses group. CONCLUSIONS There was a higher usage of sirolimus and corticosteroids in renal transplantation patients diagnosed with aHUS. Unfortunately, due to the rarity of this disease, the sample size was small (n=14). Despite the small sample size, this data analysis throws light on the relationship between aHUS and immunosuppressive agents in renal transplant recipients, although we still have much to learn.