Search

Purpose: To provide an overview of pre-selected emerging arboviruses (arthropod-borne viruses) that cause ocular inflammation in humans. Methods: A comprehensive review of the literature published between 1997 and 2023 was conducted in PubMed database. We describe current insights into epidemiology, systemic and ocular manifestations, diagnosis, treatment, and prognosis of arboviral diseases including West Nile fever, Dengue fever, Chikungunya, Rift Valley fever, Zika, and Yellow fever. Results: Arboviruses refer to a group of ribonucleic acid viruses transmitted to humans by the bite of hematophagous arthropods, mainly mosquitoes. They mostly circulate in tropical and subtropical zones and pose important public health challenges worldwide because of rising incidence, expanding geographic range, and occurrence of prominent outbreaks as a result of climate change, travel, and globalization. The clinical signs associated with infection from these arboviruses are often inapparent, mild, or non-specific, but they may include serious, potentially disabling or life-threatening complications. A wide spectrum of ophthalmic manifestations has been described including conjunctival involvement, anterior uveitis, intermediate uveitis, various forms of posterior uveitis, maculopathy, optic neuropathy, and other neuro-ophthalmic manifestations. Diagnosis of arboviral diseases is confirmed with either real time polymerase chain reaction or serology. Management involves supportive care as there are currently no specific antiviral drug options. Corticosteroids are often used for the treatment of associated ocular inflammation. Most patients have a good visual prognosis, but there may be permanent visual impairment due to ocular structural complications in some. Community-based integrated mosquito management programs and personal protection measures against mosquito bites are the best ways to prevent human infection and disease. Conclusion: Emerging arboviral diseases should be considered in the differential diagnosis of ocular inflammatory conditions in patients living in or returning from endemic regions. Early clinical consideration followed by confirmatory testing can limit or prevent unnecessary treatments for non-arboviral causes of ocular inflammation. Prevention of these infections is crucial. [ABSTRACT FROM AUTHOR]

To compare visual outcomes, ocular complications and therapies for patients with scleritis-associated intraocular inflammation (SAI) and patients with isolated scleritis (IS). A total of 52 patients (36 with SAI and 16 with IS) were reviewed. Mean age (standard deviation) at presentation was 48.4 years old (± 15.4) in the SAI group and 53 years old (± 17.1) in the IS group (p =.37). Visual acuity was worse at presentation and last visit for patients with SAI compared to IS (p =.04). Patients in the SAI group developed greater posterior segment complications than in the IS group (p =.002). Scleritis with intraocular inflammation was associated with a higher rate of visual morbidity compared to isolated scleritis. More aggressive management strategies may be needed for patients who present with scleritis associated with inflammation. [ABSTRACT FROM AUTHOR]

We provide an updated review of pre-selected RNA viruses causing ocular inflammation in humans. RNA viruses such as coronaviruses and arboviruses are reviewed elsewhere. A Google Scholar search was conducted to identify recent publications on ocular inflammation caused by the RNA viruses specified here. Human RNA viruses target a wide range of ocular tissues from the anterior to the posterior. Influenza, measles and mumps cause anterior segment manifestations including conjunctivitis and keratitis, while retinitis and optic neuritis may be seen posteriorly. Newcastle disease and RSV cause conjunctivitis, whereas HIV causes characteristic anterior uveitis. Cataracts, microphthalmos, and iris abnormalities are common in congenital Rubella, while Rubella virus is associated with Fuchs uveitis syndrome. Newer technologies make it possible to detect more than one pathogen if present simultaneously. RNA viruses may produce significant ocular morbidity, and care should be taken to investigate ocular symptoms during disease outbreaks. [ABSTRACT FROM AUTHOR]

To report the cytopathology of vitreous biopsy samples in patients with acute retinal necrosis (ARN) who underwent pars plana vitrectomy (PPV). We also describe two patients with unique clinical courses, cytopathologic findings, and immune response A retrospective review of patients with ARN who developed retinal detachment (RD) and underwent PPV from 22011 to 2019 at the Emory Eye Center was performed to assess cytopathology findings of vitreous biopsy samples. Patient demographics and laboratory testing including aqueous humor PCR for viral pathogens were recorded. Additional clinical details abstracted included intravitreal injections, surgical procedures, and vitreous cytopathological reports including immunohistochemistry findings. Fourteen eyes of 12 patients with RD were reviewed. Ten eyes showed HSV DNA (71%) and 4 demonstrated VZV DNA (29%). All eyes received intravitreal antivirals (i.e. ganciclovir or foscarnet) with a median of 8.5 intravitreal injections per eye. Diagnoses prompting PPV included tractional RD in 14 eyes (100%), rhegmatogenous RD in 8 eyes (57%), vitreous hemorrhage in 4 eyes (29%) and vitreous opacity in 4 (29%). Ophthalmic pathology reports showed lymphocyte populations in 10 eyes (71%) with a CD3 + T-cell predominance in two patients where immunohistochemistry of CD3+ and CD20+ for T- and B-cell populations was performed. Observed immune cell populations included macrophages or histiocytes (11 eyes, 79%) and polymorphonuclear cells in 4 eyes (29%). Initial median VA was 2.5 (IQR 2.0–3.0) and improved to 2.0 (IQR 1.48–3.00, p =.48) at 6-months and 1.8 (IQR 1.2–3.0, p =.45) at 12 months follow-up. Our cohort of ARN patients undergoing PPV show a spectrum of immunologic findings with the majority demonstrating a lymphocytic response. Histiocytes, macrophages, and PMNs were also observed. Cytopathologic and immunologic studies suggest that both innate and adaptive immunity are responsible for the clinical disease findings observed in ARN. The variability of the response to treatment in patients with ARN may reflect patient-to-patient differences in their antigen-specific immune response. Understanding the immunologic response associated with ARN may provide valuable information regarding the dosing and timing of treatment. [ABSTRACT FROM AUTHOR]

To present a patient with Rosai-Dorfman Disease (RDD), a histiocytic proliferative disorder typified by lymphadenopathy with rare ocular manifestations, who developed panuveitis that responded to pegylated interferon. Descriptive case report of a patient with RDD with multi-organ involvement including ocular manifestations including bilateral panuveitis with choroidal masses. A 54-year-old African American woman with known systemic RDD of the breast, lung, and gastrointestinal tract presented with panuveitis with choroidal masses in both eyes. Her systemic and ocular disease initially responded well to oral and topical steroid therapy. Later, however, her systemic disease progressed with multiple muscular and bony lesions. Systemic therapy was switched to pegylated interferon, a cytokine with antiviral, antitumor and immunomodulatory activity. After 14 months of therapy with pegylated interferon, the patient's systemic and ocular disease stabilized. Rosai-Dorfman disease may be complicated by panuveitis and choroidal masses that may respond to pegylated interferon with stabilization of systemic and ocular manifestations. A multi-disciplinary approach is essential given the unique diagnostic and management challenges of RDD. [ABSTRACT FROM AUTHOR]

To present a case of refractory systemic lupus erythematosus (SLE)-associated retinal vasculitis that responded to the anti-tumor necrosis factor (TNF)-alpha inhibitor adalimumab as corticosteroid-sparing therapy. Descriptive case report of a patient with SLE with retinal vasculitis complicated by an ischemic retinal vein occlusion and cystoid macular edema. A 30-year-old female patient with a history of SLE presented with retinal vasculitis and an ischemic, branch retinal vein occlusion with macular edema in the left eye. Oral corticosteroid was administered along with mycophenolate mofetil (MMF) as a corticosteroid-sparing agent. Despite MMF therapy, the patient developed an exacerbation of her vasculitis with the involvement of both eyes. Adalimumab was initiated with a resultant resolution of retinal vasculitis as a corticosteroid-sparing strategy with over 2 years of follow-up. Anti-TNF-alpha therapy with adalimumab may be effective as a corticosteroid-sparing agent in select patients with ocular inflammation associated with SLE. [ABSTRACT FROM AUTHOR]

Macular edema due to noninfectious uveitis is a sight-threatening complication that is routinely treated with corticosteroids. Triamcinolone acetonide injectable suspension for suprachoroidal use (Xipere™) is an alternative treatment option for patients with noninfectious uveitis-associated macular edema. This review describes the recently FDA-approved triamcinolone acetonide injectable suspension that can be injected into the suprachoroidal space. This physiological space is between the sclera and choroid. This allows for therapeutic targeting of the retina and choroid. This review highlights published clinical trials for this novel drug preparation. Suprachoroidal administration of triamcinolone acetonide has shown improvement in vision and inflammation in studies with noninfectious uveitis-associated macular edema. This unique delivery method suggests the potential to decrease side effects of anterior segment exposure such as glaucoma and cataract, but head-to-head trials are needed for further study of safety and efficacy. Additionally, there are promising prospective studies underway for utilization of the suprachoroidal space for other diseases including macular degeneration, diabetic macular edema, and ocular tumors. [ABSTRACT FROM AUTHOR]

Introduction: Juvenile idiopathic arthritis (JIA) is the most common pediatric rheumatic disease associated with uveitis. JIA-associated uveitis (JIA-U) is typically asymptomatic, chronic, and can lead to sight-threatening complications. This review will provide approaches to improve the diagnosis and outcomes of children with JIA-U. Areas covered: We will provide updates on risk factors for uveitis onset and ocular complications, improvements in the ophthalmic screening schedule for uveitis detection, and potential strategies to advance the diagnosis and monitoring of JIA-U using advanced ophthalmic imaging and diagnostic equipment and laboratory biomarkers. Expert opinion: There is a lack of high-quality research in JIA-U and few randomized controlled trials, underscoring the urgent need for further studies in this population. Early uveitis diagnosis combined with timely and appropriate treatment can improve visual outcomes. Improved knowledge of uveitis pathogenesis, risk factors for uveitis onset, measurement of uveitis outcomes, and optimal treatment are crucial. [ABSTRACT FROM AUTHOR]

Introduction: Pediatric uveitis comprises a range of ocular inflammatory diseases that may lead to vision impairment. The impact on visual functioning over the lifetime horizon can be substantial, underscoring the importance of appropriate ophthalmic evaluation, diagnostic testing and treatment. This review focuses on the anatomic classification, laboratory diagnosis, associated systemic diseases, and management of pediatric uveitis. Areas covered: A review of the literature was performed to synthesize our current understanding of the anatomic classification of pediatric uveitis, disease epidemiology, and management principles. We also review important corticosteroid-sparing strategies including non-biologic and biologic agents given their key role in the treatment of pediatric uveitis. Recent advances in the assessment of vision-related quality-of-life using the Effects of Youngsters' Eyesight on Quality of Life (EYE-Q) instrument are discussed. Expert opinion: Pediatric uveitis can lead to long-term vision impairment if not appropriately screened and treated. While topical and systemic corticosteroids are useful for the acute treatment of uveitis, the disease chronicity of many pediatric uveitis syndromes often warrants early escalation of therapy to immunosuppressive medications. Future directions include an improved understanding of risk factors for uveitis and better metrics to evaluate the impact of disease on vision-related quality-of-life of pediatric uveitis patients. [ABSTRACT FROM AUTHOR]

Introduction: The largest Ebola virus (EBOV) outbreak occurred from 2013 to 2016 in West Africa and consequently resulted in the largest cohort of Ebola virus disease (EVD) survivors to date. Ocular disease is among the most common sequelae reported in EVD survivors. This review discusses the prevalence, manifestations, pathogenesis, diagnosis, and management of EVD-related ocular disease. Areas covered: An extensive review of the literature was performed to detail the prevalence and manifestations of EVD-related ocular disease. We also review current eye screening and treatment strategies and our current understanding and approach to invasive ophthalmic procedures including surgery. Expert opinion: The ocular sequelae of EVD can lead to vision impairment or blindness, if untreated. Keys to the prevention of such an outcome include timely evaluation and access to appropriate ophthalmic care. The persistence of EBOV in the eye and other immune-privileged sites is the subject of ongoing investigation, but should not be a barrier to care if appropriate screening and biosafety measures are taken. Improved understanding of the pathogenesis of this condition and ongoing clinical care is needed for EVD survivors at-risk for ocular complications. [ABSTRACT FROM AUTHOR]

In the wake of the international Ebola virus disease (EVD) outbreak from 2014 to 2016, thousands of EVD survivors are at-risk of ophthalmic manifestations, as well as systemic sequelae including arthralgias, abdominal pain, psychosocial stressors, and risk of viral persistence in immune-privileged organs. Ophthalmic manifestations in EVD survivors include a spectrum of disease ranging from anterior uveitis to panuveitis, which confers a high risk of vision impairment and blindness, particularly given the access and resource limitations for ophthalmic subspecialty care in West Africa. Clinical studies in EVD survivors, animal models of EVD and translational investigation, have provided early insight into eye disease pathogenesis. Specifically, ocular inflammation recently observed in EVD survivors is thought to involve direct viral infection, inflammation, and tissue edema. Future research is needed to understand the timing of uveitis onset and management strategies, including the role of antiviral and anti-inflammatory therapies. [ABSTRACT FROM AUTHOR]

Purpose: To report the novel use of combined intravitreal and systemic antibiotic therapy in a patient with syphilitic panuveitis and discuss the management of ocular syphilis.Methods: Case report Results: A 45-year old heterosexual male with human immunodeficiency virus (HIV) presented with 1 month of blurry vision in both eyes. Clinical examination revealed a bilateral panuveitis. The patient denied history of genital lesions or rash, but did complain of difficulty hearing bilaterally. Treponemal EIA was positive, the RPR titer greater than 1:512 dilution, and CSF VDRL 1:4. A diagnosis of neurosyphilis and ocular syphilis was made based on the clinical and laboratory findings. The patient was admitted for systemic intravenous antibiotic therapy, but was noted to have a penicillin allergy. Intravitreal ceftazidime was promptly administered bilaterally to achieve treponemacidal levels of antibiotic therapy. After penicillin desensitization protocol, the patient received 14 days of intravenous penicillin with clinical resolution.Conclusions: There are increasing reports of ocular syphilis in the United States and delay in diagnosis and management can lead to severe visual impairment and blindness. We report the first case of adjunct intravitreal antibiotic therapy in a penicillin allergic patient. As ocular syphilis is a form of bacterial endophthalmitis, combination intravitreal and systemic antibiotics may be considered. [ABSTRACT FROM AUTHOR]

An introduction is presented in which the editors discuss various reports within the issue on topics including utilization of sustained-release corticosteroids for treating uveitis, dexamethasone (DEX) intravitreal implant, and triamcinolone acetonide (TA).

Purpose: To compare the differences in vision and health-related quality of life (HRQOL) of individuals with ocular and non-ocular sarcoidosis; and to examine the impact of specific demographic and clinical factors on the noted differences. Methods: A cross-sectional study using non-randomized prospective cohort was conducted at the National Eye Institute (protocol number: 06-EI-0239, NCT00379275) from August 31, 2006 until November 15, 2007. Each participant completed vision and HRQOL questionnaires, the Sarcoidosis Health Questionnaire (SHQ) and the National Eye Institute Visual Function Questionnaire (NEI-VFQ), along with a demographic/environmental exposure survey. Clinical data were collected through an ophthalmic exam as part of the research protocol. Results: The study enrolled 75 biopsy-proven and 20 clinically presumed sarcoidosis participants which were divided into two cohorts, ocular (N = 60) and non-ocular groups (N = 35). The ocular group had significantly lower ( P < 0.01) total NEI-VFQ scores compared to the non-ocular group. Multiple linear regression analysis showed that participants with ocular sarcoidosis who had an annual household income of < $50,000 ( P < 0.01) had significantly lower total SHQ scores while participants with ocular sarcoidosis whose visual acuity was 20/100 or worse had significantly lower total NEI-VFQ scores ( P = 0.03). Conclusions: Ocular involvement impacts both overall and vision-related quality of life among sarcoidosis patients. Lower economic status appears to have a significant impact on the quality of life of sarcoidosis patients. Assessment of visual function and general health status provide pertinent information for individuals with sarcoidosis and should be included in their care to assess burden of their disease on their quality of life. [ABSTRACT FROM AUTHOR]

Purpose: To report an unusual case of granulomatous sclerochoroiditis. Design: Interventional case report. Methods: A patient with ANCA-associated granulomatous vasculitis presented with nodular necrotizing scleritis, which was recalcitrant to multiple systemic immunosuppressive therapies and progressed to a blind painful eye, which was enucleated. Results: Histopathology revealed extensive occlusive vasculitis, diffuse T- and B- cellular infiltration, and lymphoid granulomatous formation. Enhanced MHC class II antigens, adhesion molecules, and Fas (CD95) and FasL (CD95L) were detected in the lesion. Conclusion: Granulomatous sclerochoroiditis with aggressive immune reaction can be a complication of ANCA-associated granulomatous vasculitis. [ABSTRACT FROM AUTHOR]

The pathogenic mechanisms underlying uveitis syndromes continue to be evaluated using animal models and in the clinical setting. As the complex interactions between ocular immune cells, proinflammatory cytokines, chemokines and cellular adhesion molecules are uncovered, targeted therapies directed against these immune mediators will continue to be developed. Traditional immunosuppressive medications, such as corticosteroids and steroid-sparing immunomodulatory agents, have demonstrated efficacy in the treatment of uveitic syndromes, but side effects and drug toxicities often limit the use of these medications. The biologic agents, a newer class of medications, target specific immune pathways and have demonstrated efficacy in rheumatologic, dermatologic and neurologic conditions. Biologic therapies (e.g., TNF-α inhibitors and IL-2 receptor inhibitor) targeting ocular immune cell surface receptors, cytokines and chemokines continue to be developed and have shown promise in the treatment of uveitis and ocular inflammatory diseases. Clinical and basic aspects of monoclonal antibody therapy for uveitis are presented in this review. Additional studies are needed to further evaluate the role of monoclonal antibodies in the therapeutic armamentarium for uveitis. [ABSTRACT FROM AUTHOR]

PurposeMethodsResultsConclusionDue to lack of large randomized clinical trials to determine best practices in treating acute retinal necrosis (ARN), there is not a clear consensus amongst ophthalmologists on how to best manage this potentially blinding condition. The aim of this study is to survey common practice patterns and analyze the factors that affect ophthalmologists’ management of ARN.An anonymous survey was distributed to uveitis and retina specialists who are members of the American Uveitis Society (AUS) via email to query practice patterns regarding ARN. The survey included 22 questions with an additional 10 questions based on response. Survey question topics included demographic information, diagnostic testing, antiviral therapy, corticosteroids, and surgical procedures.67 surveys were included for analysis. Most respondents (87%) always or frequently obtain intraocular aqueous fluid for diagnostic PCR testing. The majority of respondents would administer intravitreal antiviral injections to a unilateral immunocompetent ARN patient (67%), but would be even more likely to do so for a bilateral immunosuppressed ARN patient (87%). Respondents tend to treat ARN with systemic rather than local corticosteroids, with the majority (63%) of respondents initiating corticosteroid treatment 48 hours after treatment. Most respondents (79%) never perform a vitrectomy to manage ARN unless the patient has a retinal detachment or tear. The majority (63%) rarely or never perform prophylactic laser barricade, but may consider laser treatment if there is extensive retinal involvement.Current practice patterns for diagnosis and management of ARN among AUS members generally align with the suggested practices outlined by the American Academy of Ophthalmology. [ABSTRACT FROM AUTHOR]

Purpose: To describe an atypical case of primary intraocular lymphoma (PIOL) with rapid progression leading to vision loss and death. Methods: A 71-year-old woman with a history of PIOL presented with a sudden decline in vision. MRI, CSF cytology, and ocular examination 2 months prior were unremarkable. Results: Repeat MRI of the brain and orbits at this time revealed a 0.8 × 2.4-cm lesion of the left choroid plexus. LP revealed rare atypical B cells. The patient rapidly deteriorated and died a month later. Conclusion: Recognition of the potential for rapid progression and mortality in patients with PIOL is essential. [ABSTRACT FROM AUTHOR]