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.

Demyelinating plaque-associated uveitis.

PURPOSE: To report the clinical and fluorescein angiographic (FA) features of demyelinating plaque-associated uveitis (DPU), a subset of uveitis in which patients have demyelinating plaques on the brain/cervical magnetic resonance image (MRI) but do not meet the criteria for multiple sclerosis (MS). METHODS: In this retrospective observational study, Persian Patients were diagnosed with DPU and included if (1) they never satisfied the MS criteria, (2) all other possible etiologies were excluded, and (3) they were followed for at least 2 years. RESULTS: After a median follow-up of 3 years (interquartile range, 2.0-5.3), 8 out of 40 (20%) patients diagnosed with DPU were excluded as they subsequently met the MS criteria. Of remaining 32 patients studied, the mean age was 36.3±9.9 (range 20-56 years), and 30 (93.8%) were female. Twenty-four (75.0%) showed bilateral involvement and 27 (84.4%) had insidious-chronic course. Uveitis was classified as intermediate (with or without anterior uveitis) in 29 (90.6%) and isolated anterior in 3 (9.4%) patients. Nine (28.1%) patients had at least one systemic neurological complaint. Ocular findings were: granulomatous keratic precipitates in 43/44 (97.7%) eyes; snowballs in 25/52 (48.1%) eyes; snowbanks in 4/52 (7.7%) eyes; cystoid macular edema in 20/56 (35.7%) eyes; and optic neuritis in 5/56 (8.9%) eyes. Visual acuity was ≥ 20/40 in 39 eyes (69.6%) at presentation which improved to 46 eyes (81.2%) at 2-year follow up. The two most frequent findings in FA were optic disc leakage/staining in 44/52 (81.5%) eyes, and peripheral retinal perivascular leakage in 39/52 (76.9%) eyes, which in 14/52 (26.9%) eyes extended beyond the equator. CONCLUSION: DPU usually presents as a bilateral chronic granulomatous intermediate and, less often, isolated anterior uveitis, especially in females. Most are neurologically asymptomatic. Visual outcome is generally favorable. In FA, peripheral retinal perivascular leakage is common. DPU patients have an increased tendency to develop MS and should be prohibited from anti-TNF treatment.

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.

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.

Prevalence, clinical profile, investigations, and visual outcome of sarcoid intermediate uveitis in a tertiary eye care center in South India.

Purpose: To report the prevalence, clinical profile, investigations, and visual outcomes of sarcoid intermediate uveitis in a tertiary eye care center in South India. Methods: Retrospective, observational case series. Records of 29 patients with sarcoid intermediate uveitis were retrieved. Complete ophthalmic evaluation and systemic examination by a pulmonologist with appropriate laboratory investigations were done. Results were analyzed using SPSS software. Results: Mean age group was 42.14 ± 11.31 years. The bilateral presentation was more common and females were more affected than males. Anterior chamber cells and flares in 22.4% of cases (N = 11 eyes), posterior synechiae in 20.4% (N = 10 eyes), and both small and mutton fat keratic precipitates in 14.2% of cases (N = 7 eyes) were noted; only one eye had Busacca nodules similar to other granulomatous uveitis. Cystoid macular edemas were present in three eyes. Treatment with oral steroids and systemic immunosuppression resulted in good visual recovery. The mean presenting visual acuity in right and left eye were 0.2 and 0.3, respectively. The mean final visual acuity in right and left eye was 0.1 and 0.3, respectively. Conclusion: Sarcoid intermediate uveitis is quite common in a tuberculosis endemic country like India. A complete review of systems with appropriate investigations is essential to prevent visual complications.

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.

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.

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®).

Risk of Cataract in Intermediate Uveitis.

PURPOSE: To determine the incidence of and predictive factors for cataract in intermediate uveitis. DESIGN: Retrospective cohort study. METHODS: Patients were identified from the Systemic Immunosuppressive Therapy for Eye Diseases Cohort Study, in which medical records were reviewed to determine demographic and clinical data of every eye/patient at every visit at 5 participating US tertiary care uveitis centers. The primary outcome was development of vision-compromising cataract as defined by a decrease in visual acuity to 20/40 or less, or requiring cataract surgery. Survival analysis assessed visually defined cataract to avoid bias due to timing of surgery vis-à-vis inflammatory status. RESULTS: Among 2,190 eyes of 1,302 patients with intermediate uveitis, the cumulative incidence of cataract formation was 7.6% by 1 year (95% confidence interval [CI] = 6.2%-9.1%), increasing to 36.6% by 10 years (95% CI = 31.2%-41.6%). Increased cataract risk was observed in eyes with concurrent anterior uveitis causing posterior synechiae (hazard ratio = 2.68, 95% CI = 2.00-3.59, P < .001), and in eyes with epiretinal membrane formation (hazard ratio = 1.54, 95% CI = 1.15-2.07, P = .004). Higher dose corticosteroid therapy was associated with significantly higher incidence of cataract, especially time-updated use of topical corticosteroids ≥2 times/d or ≥4 periocular corticosteroid injections. Low-dose corticosteroid medications (oral prednisone 7.5 mg daily or less, or topical corticosteroid drops <2 times/d) were not associated with increased cataract risk. CONCLUSIONS: Our study found that the incidence of clinically important cataract in intermediate uveitis is moderate. The risk is higher with markers of severity and with higher doses of corticosteroid medications, the latter being potentially modifiable.

Intermediate Uveitis Associated with Tattooing of Eyebrows as a Manifestation of Systemic Sarcoidosis: Report of Two Cases.

Permanent make-up tattooing as a cosmetic procedure is gaining popularity especially among women. Although it is considered a relatively safe intervention, the ink used can rarely be a trigger of sarcoidosis. Uveitis can occur as part of this inflammatory process. In this study, we describe two ladies presented with tattoo-associated uveitis as the first manifestations of systemic sarcoidosis. They developed intermediate uveitis shortly after skin inflammation several months after permanent make-up tattooing of eyebrows. Lung involvement, high ACE levels, and negative PPD were present. Skin granuloma formation was diffuse over the area of tattoo in one patient and localized in the other one. This is the first report of uveitis following make-up tattoo.

Comparison of conventional immunosuppressive drugs versus anti-TNF-α agents in non-infectious non-anterior uveitis.

OBJECTIVE: To compare the efficacy and safety of Disease-modifying antirheumatic drugs (DMARDs) and anti-TNF-α agents in patients with non-infectious non-anterior uveitis. METHODS: Single center retrospective study including adult patients with non-infectious intermediate, posterior or pan-uveitis. Outcomes were compared between patients treated with DMARDs or anti-TNF-α agents. The primary outcome was treatment failure or occurrence of serious adverse events. Treatment failure was determined by ophthalmologic criteria. RESULTS: Seventy-three patients were included, mostly female (52%). Among them, 39 were treated with DMARDs and 34 with anti-TNF-α agents. The main uveitis causes were idiopathic (30%), birdshot chorio-retinopathy (25%), sarcoidosis (16%) and Behçet's disease (14%). The primary outcome was observed in 56% of patients treated with anti-TNF-α agents versus 59% of patients treated with DMARDs (p = 0.82). Median time to observe the primary outcome was 16 months (anti-TNF-α group) versus 21 months (p = 0.52). There was no significant difference between the two groups in terms of treatment failure, corticosteroid sparing effect, visual acuity improvement or adverse events. Earlier control of ocular inflammation was achieved with anti-TNF-α agents than with DMARDs (p = 0.006). In relapsing patients, anti-TNF-α agents allowed better corticosteroid sparing (p = 0.06). CONCLUSION: DMARDs could still be used as first-line therapy for non-infectious non-anterior uveitis after corticosteroid therapy. However, anti-TNF-α agents could be proposed as an alternative in cases of severe inflammation or initial high level of steroid dependency.

Time to disease recurrence in noninfectious uveitis following long-acting injectable fluocinolone acetonide implant.

PURPOSE: To determine the time to disease recurrence with long-acting injectable fluocinolone acetonide implant (FAi) for noninfectious intermediate, posterior, and panuveitis. METHODS: This was a retrospective study of patients with at least 12 months of follow-up who had completed a 2-year prospective, investigational new drug study with 0.18-mg FAi. Time to uveitis recurrence or cystoid macular edema (CME) occurrence was recorded. RESULTS: Twelve eyes from 12 participants (mean age 43 years, range 25-64 years) were included. Patients were followed for a mean of 34.2 months (range, 12.0-56.9 months) after completion of the prospective trial. Five eyes (42%) did not have a documented uveitis recurrence or CME occurrence. Five eyes (42%) had a uveitis recurrence with the mean time to recurrence 36.1 months (range, 22.8-61.1 months) after FAi implantation. Two eyes (16%) had CME alone, the mean time to occurrence 36.9 months (range 36.1-42.1 months). On Kaplan-Meier analysis, the estimated probability of remaining recurrence-free 36 months after FAi implantation was 0.67 (95% confidence interval, 0.34-0.86). CONCLUSIONS: Data of study participants after completing a clinical trial suggest that the injectable FAi for noninfectious uveitis can provide control for 3 years on average. These long-term data support the use of FAi to control noninfectious uveitis.

Intermediate uveitis in a child with phosphatase and tensin homolog gene mutation and Bannayan-Riley-Ruvalcaba syndrome.

Bannayan-Riley-Ruvalcaba syndrome (BRRS) is a congenital disorder characterised by macrocephaly, multiple hamartomas, lipomas, and pigmented macules of the glans penis. Intermediate uveitis is characterised by chronic inflammatory cells aggregates on the pars plana (snowbanks) and within the vitreous cavity (snowballs). We describe what we believe to be the first case of intermediate uveitis associated with BRRS. Early examination under anaesthesia should be considered in the management of young children diagnosed with this syndrome in order to provide appropriate ocular evaluation, treatment and follow-up. Further research is needed to establish a better understanding of the ophthalmic manifestations of this syndrome.

Intermediate Uveitis as the Initial and Only Presentation of Syphilis

We report a patient with unilateral syphilitic intermediate uveitis without dermatological, neurological, or any systemic involvement. He presented to our clinic with complaints of eye floaters and worsening visual acuity in the left eye. He had intermediate uveitis and cystoid macular edema in that eye and both venereal disease research laboratory and microhemagglutination assay for Treponema pallidum serological tests were confirmatory for syphilis. Ocular manifestations of syphilis have variable presentations, and it should be considered when diagnosing unexplained ocular inflammatory diseases, even if the patient's recent history and systemic evaluation are not compatible.

Swept-source optical coherence tomography angiography reveals vascular changes in intermediate uveitis.

PURPOSE: To evaluate retinal and choroidal vascular changes in patients with intermediate uveitis with/without concomitant retinal vasculitis using wide field swept-source optical coherence tomography angiography (OCTA). METHODS: In this study consecutive patients with intermediate uveitis ± vasculitis and healthy age-matched controls were evaluated using central 3 × 3 mm OCTA scans and montage scans. Differences among the groups as well as the association between central changes assessed by 3 × 3 scans and wide field OCTA were evaluated. RESULTS: 93 eyes of 58 patients with intermediate uveitis and 33 healthy age-matched controls were included. The presence of a cystoid macular oedema (CME) was associated with capillary non-and reduced perfusion of the superficial capillary plexus (SCP) (p = 0.03 and 0.02, respectively), and deep capillary plexus (DCP) (p = 0.02 and p ≤ 0.0001, respectively) of the 3 × 3 mm scans. The raw length, circularity and size of foveal avascular area (FAZ) significantly differed among the three groups. (p = 0.01, p = 0.045 and p = 0.004, respectively). Multivariable regression analysis revealed that the presence of epiretinal membranes (ERM) and CME rather than the disease entity contributed to the changes of FAZ size (R2  = 0.15, p = 0.0003) The mean vessel density (VD) of the SCP significantly differed among the three groups (intermediate uveitis with concomitant vasculitis: 16.8 ± 3.8 mm-1 vs intermediate uveitis only: 15.6 ± 4.4 mm-1 versus control: 18 ± 3.5 mm-1 , p = 0.046). Multivariable regression analysis showed that the presence of CME rather than the disease entity impacted vessel density of SCP (R2  = 0.1, p = 0.016). There was no association between peripheral non- or reduced perfusion of the wide field OCTA slabs of the SCP and DCP and the VD of the 3 × 3 slabs. CONCLUSION: Although patients with intermediate uveitis and vasculitis present with reduced central vessel density compared to healthy age-matched controls, these changes are presumably contributed to the presence of CME.

Visual and Clinical Outcome of Macular Edema Complicating Pediatric Noninfectious Uveitis.

PURPOSE: To investigate the clinical course and visual outcome of macular edema (ME) in pediatric patients with chronic noninfectious uveitis. DESIGN: Retrospective case series. METHODS: The databases of the uveitis clinics of 4 tertiary medical centers in Israel and the UK were searched for all children treated for uveitic ME in the years 2005-2015. Data were collected from the medical records as follows: demographics, diagnosis, visual acuity, clinical and imaging findings, and treatment given specifically for ME. Findings at baseline and at 3, 6, 12, and 24 months were evaluated. RESULTS: The cohort included 25 children (33 eyes) of mean age 8.5 ± 3.4 years. The most common diagnosis was intermediate uveitis, in 14 children (7 idiopathic, 7 pars planitis). Uveitis was active at ME diagnosis in 28 eyes (84.8%). Median duration of follow-up was 48 months. Median time to resolution of ME was 6 months, with complete resolution in 25 eyes (75.8%) by 24 months. Baseline visual acuity was ≥20/40 in 8 eyes (24.2%), increased to 57.6% at 3 months (P < .0001), and remained stable thereafter. Treatment regimens included corticosteroids (systemically and/or locally), immunosuppression, and biologic therapies. No correlation was found between outcome and either structural characteristics of ME or specific treatment strategy. CONCLUSIONS: The prognosis of pediatric uveitic ME is favorable despite its chronic course. Larger randomized controlled trials are needed to define differences among treatment regimens.

Ultra-Widefield Fluorescein Angiography in Intermediate Uveitis.

Purpose: To examine associations between pattern of vascular leakage on ultrawide-field fluorescein angiography (UWFFA) and visual acuity, cystoid macular edema (CME), and inflammatory activity in intermediate uveitis. Methods: Single center cross-sectional, retrospective review of medical records, spectral domain optical coherence tomography (SD-OCT) and angiographic images of intermediate uveitis patients who underwent UWFFA over a 12-month period. Results: Forty-one eyes from 24 patients were included. Twelve eyes (29%) exhibited peripheral leakage, 26 eyes (64%) had diffuse leakage and three eyes (7%) had no leakage. Diffuse leakage was associated with 0.2 logMAR worse visual acuity than peripheral leakage (p = 0.02). There was no statistically significant difference in the odds of having CME when diffuse leakage was compared to peripheral leakage. Conclusion: UWFFA identifies retinal vascular pathology in intermediate uveitis not present on clinical examination. Diffuse retinal vascular leakage was associated with worse visual acuity when compared to peripheral and no leakage patterns.