Vasoproliferative Tumor Secondary to Sarcoidosis-Associated Intermediate Uveitis.

We report the visual and clinical outcomes of a middle-aged woman who presented with exudative retinal detachment (ERD) secondary to a vasoproliferative tumor (VPT) in an eye with sarcoidosis-associated intermediate uveitis. A 55-year-old woman previously diagnosed with sarcoidosis presented with decreased vision in the left eye (LE). Visual acuity in the LE was counting fingers. She had active vitritis, and a peripheral retinal vascular mass was noted in the superotemporal periphery. The mass was associated with ERD involving the posterior pole. The patient was managed with systemic and intravitreal steroids, and cyclosporine was subsequently added as a steroid-sparing agent. Because of recurrence of ERD, the patient underwent pars plana vitrectomy, and cryotherapy and laser photocoagulation were applied to the VPT. Two months postoperatively, visual acuity in the LE improved to 6/10. There was marked regression of the VPT and total resolution of the ERD. In conclusion, we report a favorable visual and clinical outcome in a patient with VPT-associated ERD who responded to a combination of medical therapy and surgical intervention. VPT may lead to different remote complications, so timely diagnosis of these tumors and proper management of their complications is warranted.

New onset of uveitis during infliximab treatment: A case report.

INTRODUCTION: Anti-tumor necrosis factor α (anti-TNF α) agents are an effective treatment for a variety of inflammatory and autoimmune diseases. In ophthalmology anti-TNF α began to emerge as a possible therapy for non-infectious uveitis, paradoxically their administration may result in the onset or recurrence of inflammatory eye disease such as uveitis. We reported a case of new onset of bilateral anterior and intermediate uveitis in a patient with rheumatoid arthritis (RA) while being treated with infliximab and we performed a review of literature. OBSERVATION: A 25-year-old female with RA under infliximab, presented with bilateral blurred vision. Anterior segment examination demonstrated retrodescmetic fine precipates, 1+ cells in the anterior chamber on both eyes. The fundus examination was difficult because of the vitritis. Fluorescein angiography demonstrated mild optic disc edema, and bilateral diffuse peripheral fern leaf cappilaritis. Optical coherence tomography showed severe cystoid macular edema bilaterally. The diagnosis of bilateral anterior and intermediate uveitis caused by infliximab was retained after exclusion of infectious and autoimmune aetiologies. She was treated with corticosteroid with good visual outcome. CONCLUSION: In our case, new onset of uveitis may be considered as paradoxical effect of anti-TNF α therapy. Rheumatologists and ophthalmologists should be aware of this effect. Careful monitoring of patients under infliximab is necessary for appropriate diagnosis and early treatment.

Uveitis in the Setting of Co-Existing Systemic Sarcoidosis and Multiple Sclerosis.

We report a case of intermediate uveitis in the setting of both systemic sarcoidosis and multiple sclerosis. A 68-year-old female was diagnosed with bilateral granulomatous intermediate uveitis and cystoid macular edema. Initial systemic work-up was unrevealing. The uveitis was treated successfully with local corticosteroid injections. Eighteen months after presentation, the patient developed new systemic symptoms. Additional testing revealed systemic lymphadenopathy, with biopsy showing non-caseating granulomas, leading to a diagnosis of sarcoidosis. However, MRI of the brain and spinal cord along with cerebrospinal fluid analysis was consistent with MS. The management of the uveitis and systemic inflammation was co-managed by ophthalmology, neurology, and rheumatology, and eventually controlled with leflunomide and rituximab. Patients can rarely have co-existing systemic sarcoidosis and multiple sclerosis. Although challenging to diagnose, radiographic findings and cerebrospinal fluid analysis can be helpful to differentiate multiple sclerosis and neurosarcoidosis. Management of these patients requires coordination between multiple specialties.

Pediatric Pars Planitis: A Review.

PURPOSE: To provide an overview of pediatric pars planitis. METHODS: Narrative literature review. RESULTS: Pars planitis refers to the idiopathic subset of intermediate uveitis in which there is vitritis along with snowball or snowbank formation occurring in the absence of an associated infection or systemic disease. It is thought to be a T-cell mediated disease with a genetic predisposition. Pars planitis accounts for 5-26.7% of pediatric uveitis cases. Presentation is commonly bilateral but asymmetric, often with insidious onset of floaters and blurred vision. Although pars planitis is known to be a benign form of uveitis in most cases, severe complications secondary to chronic inflammation may arise, with cystoid macular edema being the most common cause of visual morbidity. Mild vitritis in the absence of symptoms, vision loss, or macular edema may be observed. Patients with severe vitritis and/or associated vision-threatening complications require prompt aggressive treatment. A stepladder approach including corticosteroids, immunosuppressive agents, anti­tumor necrosis factor­alpha and pars plana vitrectomy and/or laser photocoagulation is the most commonly used method for treatment of pars planitis. CONCLUSION: Timely diagnosis and adequate treatment of pediatric pars planitis and associated complications are crucial in order to improve visual outcomes.

[Intermediate and Posterior Uveitis - Classification, diagnostics, complications, and therapeutic algorithms]. / Intermediäre und posteriore Uveitis ­ Teil 1.

Intermediate and posterior uveitis describes a broad variety of different types of intraocular inflammation. Before starting treatment of intermediate or posterior uveitis, a differentiation between infectious or non-infectious uveitis must always be made. Pathognomonic symptoms do not exist, visual loss and vitreous floaters are the most common symptoms. The indication for therapy is influenced by the anatomical localization, the degree of inflammation, an association, complications and the activity of the inflammation. In addition to clinical ophthalmological standard examination, angiography and OCT are the most important investigations to classify and assess the course of inflammation. Macular edema is the most common complication of intermediate or posterior uveitis and should be treated at first onset, recurrence, or worsening. Oral, intravenous, or intravitreal corticosteroids are usually the primary therapy for intermediate or posterior uveitis. Systemic immunosuppression is indicated after steroid failure in non-infectious uveitis.

Correlation between Morphological Characteristics of Macular Edema and Visual Acuity in Young Patients with Idiopathic Intermediate Uveitis.

Background and Objectives: Macular edema (ME) is a common complication of intermediate uveitis (IU). It is often responsible for a decrease in visual acuity (VA). Three distinct patterns of macular edema have been described in intermediate uveitis, namely, cystoid macular edema (CME), diffuse macular edema (DME), and serous retinal detachment (SRD). The current study aims to describe the characteristics of macular edema in young patients with idiopathic intermediate uveitis and to correlate its features with VA using spectral domain optical coherence tomography (SD-OCT). Materials and Methods: A total of 27 eyes from 18 patients with idiopathic IU complicated by ME were included in this retrospective study. All patients underwent SD-OCT; data were gathered at the onset of ME. Best-corrected VA (BCVA) was correlated with the morphological features of ME. Results: BCVA was negatively correlated with Ellipsoid Zone (EZ) disruption (p = 0.00021), cystoid pattern (p = 0.00021), central subfield thickness (CST) (p < 0.001), and serous retinal detachment (0.037). Conclusions: In ME secondary to idiopathic IU, VA negatively correlates with Ellipsoid Zone disruption and increases in CST. Moreover, vision is influenced by the presence of cysts in the inner nuclear and outer nuclear layers and by the neuroepithelium detachment.

The Efficacy and Safety of Intravitreal Dexamethasone Implant as Anti-inflammatory Monotherapy in the Management of Tuberculosis-associated Intermediate Uveitis.

AIM: To study the long-term efficacy and safety of Ozurdex as anti-inflammatory monotherapy in the management of tuberculosis-associated intermediate uveitis (TBIU). METHODS: Retrospective analysis of eyes with TBIU that received Ozurdex as anti-inflammatory monotherapy with a follow-up of at least 1 year. RESULTS: 13 eyes (2 pseudophakic) of 11 patients were included. Mean BCVA improved from 0.65 to 0.11 at 3 months (p = .0005) and remained 0.11 at 1 year. Mean central foveal thickness improved from 452.87µ to 187.25µ at 3 months (p = .0009) and 184.62µ at 1 year in 8 eyes with CME. Mean vitreous haze improved from 2.38 to 0.11 at 3 months with no recurrences at 1 year. Mean IOP increased from 13.15mmHg to 15.53mmHg (p = .013) at 2 months and reduced to 12.46mmHg by 1 year. None required antiglaucoma medication. One eye underwent cataract surgery. The mean follow-up was 18.4 months. CONCLUSION: Ozurdex is safe and efficacious in TBIU as anti-inflammatory monotherapy in conjunction with antitubercular therapy.

Intermediate Uveitis: A Review.

PURPOSE: This review aims to provide an update on the clinical presentation, etiologies, complications, and treatment options in intermediate uveitis (IU). METHODS: Narrative literature review. RESULTS: IU affects all age groups with no clear gender predominance and has varied etiologies including systemic illnesses and infectious diseases, or pars planitis. In some instances, IU may be the sole presentation of an underlying associated condition or disease. Management of IU and its complications include administration of corticosteroids, antimetabolites, T-cell inhibitors, and/or biologics, along with surgical interventions, with varying degrees of effectiveness across literature. In particular, increasing evidence of the safety and efficacy of immunomodulatory agents and biologics has seen greater adoption of these therapies in clinical practice. CONCLUSIONS: IU is an anatomical description of uveitis, involving intraocular inflammation of the vitreous, peripheral retinal vasculature, and pars plana. Various treatment options for intermediate uveitis are currently used in practice.

Multiple Sclerosis in a Patient with Intermediate Uveitis and Juvenile Idiopathic Arthritis Treated with Adalimumab: A Case Report.

PURPOSE: To report a case of multiple sclerosis (MS) development in a patient with Juvenile Idiopathic Arthritis (JIA) and bilateral intermediate uveitis (IU) treated with Adalimumab. CASE REPORT: A 21-year-old Colombian woman diagnosed with JIA and bilateral refractory IU treated with methotrexate and Adalimumab with difficult control of the disease and multiple ocular complications. Eight years after starting Adalimumab, the patient presented paresthesia in the left upper limb. Radiologic findings in the brain and cervical spine MRI confirmed the diagnosis of MS. CONCLUSIONS: We reported the first case of MS development in a patient with JIA treated with Adalimumab and the third in a patient with noninfectious uveitis treated with anti-TNFα. It remains uncertain whether MS is secondary to anti-TNFα therapy or is linked to a polyautoimmunity phenomenon.

Long term complications and vision loss in HLA-B27 uveitis.

OBJECTIVES: To evaluate the long term complications and vision loss in HLA-B27 uveitis. METHODS: Retrospective review of subjects with HLA-B27 uveitis in a public tertiary centre between January 2008 and 2020. RESULTS: 562 HLA-B27-positive subjects (834 eyes) had mean follow-up of 9.8 years (8173.2 eye-years). Median visual acuity at ten years was 0.1 logMAR (IQR 0.0-0.1). Complications occurred in 404 eyes (48.4%): posterior synechiae (39.7%), cataract (22.1%), elevated intraocular pressure (15.5%), cystoid macular oedema (6.0%). Permanent moderate vision loss ( ≤ 0.4 logMAR) due to uveitis occurred in 14 eyes (1.7%) and severe vision loss (≤ 1.0 logMAR) in 7 eyes (0.8%). Complications were more common with older age (OR 1.017 p = 0.016), chronic inflammation (OR 5.272 p < 0.001) and intermediate uveitis (OR 5.982 p < 0.001). CONCLUSIONS: Complications are frequent in HLA-B27 uveitis, especially in older subjects, chronic inflammation and intermediate uveitis. Despite this, the majority of subjects maintain good visual prognosis.

Clinical course of HTLV-1 infection associated intermediate uveitis.

OBJECTIVE: To describe the clinical features at presentation, delivered treatment and follow-up of a case series of human T-cell lymphotropic virus type 1 (HTLV-1) associated intermediate uveitis. PATIENTS AND METHODS: Retrospective, descriptive and longitudinal study of patients with HTLV-1 associated intermediate uveitis treated at a reference ophthalmology facility in Lima, Peru, during the years 2012 to 2018. RESULTS: A total of 18 patients (28 eyes) were included, the average age at presentation was 57.3 years, 66.6% were women, and the average follow-up time was 1,280 days. The most frequent symptoms were blurred or diminished vision (78.6%) and floaters (57.1%). Best corrected visual acuity was 20/40 or better in 53.6%. The mean initial intraocular pressure was 14.95 mmHg. Keratic precipitates were observed in 50% of eyes, 17.9% were of the stellate type. The most frequent treatment was periocular corticosteroid injections (53.6%). Complications such as epimacular membrane (50%), cataract (21.4%) and glaucoma (7.1%) occurred. At the end of follow-up, only 2 eyes lost one line of vision; the final best corrected visual acuity was 20/40 or better in 85.7%, and 20/70 or better in 96.4%. Patients with both eyes affected increased from 33% at presentation to 55.5%. The course of the disease was chronic in 60.7%. CONCLUSION: HTLV-1 associated intermediate uveitis mainly occurred in patients in the second half of life, developing a chronic course and with good visual prognosis.

Review of de novo uveitis in older adults presenting to a large tertiary centre.

BACKGROUND/AIMS: The primary aim of this study was to describe the causes of de novo uveitis in individuals 60 years and older. Secondary objectives were to determine the incidence of intraocular lymphoma and the clinical predictors of lymphoma. METHODS: Retrospective chart review of all subjects presenting to the uveitis service at Auckland District Health Board (Auckland, New Zealand) between January 2006 and October 2020 RESULTS: 686 subjects (900 eyes) were aged ≥60 years at first presentation with uveitis, representing 23.4% of all subjects with uveitis during the study period. Non-infectious aetiology occurred in 631 (70.1%) eyes and infectious etiologies occurred in 269 (29.9%) eyes. The most frequent causes were idiopathic (36.3%), herpes zoster (14.8%), HLAB27 (8.7%) and sarcoidosis (4.8%). Twenty (2.2%) eyes of 13 (1.9%) subjects had a diagnosis of lymphoma. Lymphoma represented 11.2% of all intermediate uveitis. Subjects diagnosed with lymphoma did not develop posterior synechiae, epiretinal membrane, cystoid macular oedema or ocular hypertension. CONCLUSIONS: Intraocular lymphoma was uncommon in the overall cohort, but an important cause of intermediate uveitis. A diagnosis of lymphoma needs to be considered in any older subject with de novo intermediate uveitis. The lack of posterior synechiae, cystoid macular oedema, epiretinal membrane and ocular hypertension further increases the suspicion for lymphoma.

Dexamethasone Intravitreal Implant (Ozurdex) in Paediatric Patients with Non-infectious Intermediate Uveitis and Related Cystoid Macular Oedema: Evaluation of Macular Morphology and Function with Six-month Follow-up; a Deeper Role of MfERG?

PURPOSE: To evaluate the efficacy of Ozurdex implant by analyzing macular morphology and function in pediatric uveitis and related cystoid macular edema (CMO). METHODS: Main outcomes were visual acuity, mfERG and photopic ERG response, and central macular thickness. Mean values recorded at each time-point were compared to baseline and correlations between functional and anatomical parameters were evaluated. RESULTS: Resolution of intraocular inflammation and CMO was achieved in all eyes 1 month after implant without procedure or drug-related complications. Mean visual acuity and mfERG amplitude improved showing a statistically significant difference to baseline values for the first 4 months. Mean central macular thickness showed a statistically significant reduction for all follow-up time. Photopic ERG did not vary significantly. Statistically significant correlation was found between trends of visual acuity, central macular thickness, and mfERG responses. CONCLUSION: Correlation found between macular morphology and function confirms the efficacy of Ozurdex in pediatric uveitis.

Relevance of Brain MRI in Patients with Uveitis: Retrospective Cohort on 402 Patients.

AIM: To assess the diagnostic value of brain magnetic resonance imaging (bMRI) for the etiological diagnosis of uveitis and to establish predictive factors associated with its advantageous use. METHODS: Retrospective study on all patients with de novo uveitis who were referred to our tertiary hospital and who underwent a bMRI between 2003 and 2018. RESULTS: bMRI was contributive in 19 out of 402 cases (5%), among patients with a contributive bMRI, 68% had neurological signs. Univariate analysis established that neurological signs (p < .001), granulomatous uveitis (p = .003), retinal vasculitis (p = .002), and intermediate uveitis (p < .001) were all significantly associated with a contributive bMRI. Multivariate analysis confirms the significant association of neurological signs (p < .001) and intermediate uveitis (p = .01). CONCLUSION: bMRI appears to be a relevant exam in specific cases; intermediate/posterior uveitis or panuveitis accompanied by neurological signs, retinal vasculitis, or in patients older than 40, to rule out an oculocerebral lymphoma. ABBREVIATIONS: ACE: Angiotensin-Converting Enzyme; bMRI: Magnetic Resonance Imaging; CBC: Complete Blood cell Count; BMRI: Brain Magnetic Resonance Imaging; CT: Computerized Tomography; MS: Multiple Sclerosis; NS: Neurological Signs; OCL: Oculocerebral Lymphoma; RIS: Radiologically Isolated Syndrome.

Long-Term Outcomes of Pediatric Idiopathic Intermediate Uveitis.

PURPOSE: To describe the course of childhood-onset intermediate uveitis without associated systemic disease, and investigate determinants of outcomes. DESIGN: A retrospective clinical cohort study METHODS: This study was conducted in an institutional setting. A total of 125 children (221 eyes) aged 16 years and less participated. Outcomes of interest were visual acuity, severity of inflammation, and the occurrence of sight-threatening complications. Variables examined included age and clinical findings at presentation, treatment, and duration of follow-up. Multivariable analysis was undertaken to investigate potential predictors of outcomes. RESULTS: The median follow-up duration was 57 months. At presentation, best-corrected visual acuity worse than 20/160 was recorded in 11 (4.4%) eyes and significant vitreous haze (≥2+Standardisation of Uveitis Nomenclature (SUN)) in 35 (14%) eyes. Corticosteroid-sparing agents were used in 41 children (33%), with methotrexate most commonly used (27 children, 21.6%). The most frequent complications were raised intraocular pressure (n = 65; 29.4%), cataract (n = 41; 18.5%), and cystoid macular edema (n = 29; 13.1%). At the last visit, 116 (92.8%) patients achieved best-corrected vision of 20/40 or better with quiescent uveitis. The absence of the use of a steroid-sparing immunomodulatory agent was the strongest predictive factor for the development of new macular edema (odds ratio = 6.3, 95% CI = 2.3-16.9, P < .001) or glaucoma (odds ratio = 6.6, 95% CI = 2.5-17.9, P < .001) over the period of observation. CONCLUSIONS: The visual outcomes of childhood-onset idiopathic intermediate uveitis are favorable. The frequency of sight-threatening sequelae of inflammation, which confer a lifelong risk of further visual loss, is high. The use of immunomodulatory therapy is associated with a lower risk of developing macular edema and ocular hypertension.

Differential Diagnosis of Vitritis in Adult Patients.

The term "vitritis" refers to the presence of a cellular infiltration of the vitreous body, usually in the context of an intraocular inflammation, but not exclusively. Intermediate uveitis is the most prominent cause of vitritis, including infectious and auto-immune/auto-inflammatory etiologies. Corticosteroids and immunosuppressive therapies should not be started before ruling out the infectious causes of vitritis, especially in immunosuppressed individuals. Other situations can mimic intermediate uveitis such as amyloidosis and ocular tumors. Primary intraocular lymphoma should always be suspected in case of vitreous infiltrations in individuals aged over 50 years.

Unilateral intravitreal dexamethasone implant for the treatment of cystoid macular edema in intermediate uveitis / Implante unilateral de dexametasona intravítrea para tratamento de edema macular cistoide na uveíte intermediária

ABSTRACT This report aims to describe the effectiveness of a unilateral intravitreal dexamethasone implant (Ozurdex®) used for the treatment of cystoid macular edema in a patient with recurrent intermediate uveitis. Bearing in mind the adverse effects of the prolonged use of systemic corticosteroids, the objective here was to provide a less damaging form of intervention, and also to demonstrate the safety of the dexamethasone implant for patients who fail to respond to conventional treatment. In the present case, there was bilateral improvement in retinal anatomy and function with use of the unilateral intravitreal dexamethasone implant (Ozurdex®).

RESUMO Neste estudo, o objetivo foi descrever, a partir de um relato de caso, a eficácia do uso de implante de dexametasona intravítrea (Ozurdex®) unilateral, para o tratamento de edema macular cistoide, em um paciente com quadro de uveíte intermediária recorrente, visando uma terapêutica menos lesiva, diante dos efeitos colaterais do uso prolongado de corticoesteroides sistêmicos, demonstrando também a segurança desse tratamento alternativo para aqueles pacientes que se apresentam refratários a terapêutica tradicional. No caso relatado, vale ressaltar a melhora bilateral da anatomia e função retiniana com o implante unilateral de dexametasona intravítrea (Ozurdex®).

Intermediate uveitis associated with MS: Diagnosis, clinical features, pathogenic mechanisms, and recommendations for management.

Uveitis is a major cause of visual impairment and blindness among working-age adults, accounting for 10% of legal blindness in the United States. Among people with MS, the prevalence of uveitis is 10 times higher than among the general population, and because MS and uveitis share similar genetic risk factors and immunologic effector pathways, it is not clear whether uveitis is one of the manifestations of MS or a coincident disorder. This uncertainty raises several diagnostic and management issues for clinicians who look after these patients, particularly with regard to recognizing visual symptoms resulting from demyelination, intraocular inflammation, or the visual complications of disease modifying drugs for MS, e.g., fingolimod. Likewise, management decisions regarding patients with uveitis are influenced by the risk of precipitating or exacerbating episodes of demyelination, e.g., following anti-tumor necrosis factor biologic therapy, and other neurologic complications of immunosuppressive treatments for uveitis. In this review, we explore the similarities in the pathophysiology, clinical features, and treatment of patients with uveitis and MS. Based on the latest evidence, we make a set of recommendations to help guide neurologists and ophthalmologists to best manage patients affected by both conditions.

Incidence and Outcome of Uveitic Glaucoma in Eyes With Intermediate, Posterior, or Panuveitis Followed up to 10 Years After Randomization to Fluocinolone Acetonide Implant or Systemic Therapy.

PURPOSE: To evaluate long-term risk and outcomes of glaucoma in eyes with intermediate, posterior, and panuveitis managed with systemic or fluocinolone acetonide (0.59 mg, "implant") therapy. DESIGN: Prospective Follow-up of the Multicenter Uveitis Steroid Treatment (MUST) Clinical Trial Cohort. METHODS: Patients with intermediate, posterior, or panuveitis randomized to implant or systemic therapy (corticosteroid plus immunosuppression in >90%) were followed prospectively for glaucoma incidence and outcome. RESULTS: Among 405 uveitic at-risk eyes of 232 patients (median follow-up = 6.9 years), 40% (79/196) of eyes assigned and treated with implant and 8% (17/209) of eyes assigned and treated with systemic therapy (censoring eyes receiving an implant on implantation) developed glaucoma (hazard ratio [HR] = 5.9, 95% confidence interval [CI] 3.2, 10.8; P < .001). Adjustment for intraocular pressure (IOP) elevation during follow-up only partially mitigated the association of implant treatment with glaucoma incidence: HR = 3.1 (95% CI 1.6, 6.0); P = .001. Among 112 eyes of 83 patients developing glaucoma, the 5-year cumulative incidence following diagnosis of sustained (2 or more consecutive visits) worsening of mean deviation by ≥6 dB was 20% (95% CI 12%, 33%); 5-year cumulative incidence of sustained worsening of cup-to-disc ratio by ≥0.2 was 26% (95% CI 17%, 39%). CONCLUSIONS: The implant has substantially higher risk of glaucoma than systemic therapy, a difference not entirely explained by posttreatment IOP elevation. Management of IOP elevation was effective in preventing worsening of glaucoma for the large majority of cases, but even under expert clinical management, some glaucoma worsened. Uveitis cases should be monitored carefully for IOP elevation and glaucoma indefinitely.