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.

[Intermediate and Posterior Uveitis - Disease entities]. / Intermediäre und posteriore Uveitis ­ Teil 2.

Intermediate and posterior uveitis can have multiple infectious and noninfectious causes, and posterior uveitis in particular is clinically multifaceted. Some entities require prompt initiation of therapy to ensure visual prognosis. This article presents typical characteristics of intermediate and posterior uveitides and explains special features of their treatment.

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.

Clinical characteristics of intermediate uveitis in adults according to criteria of the SUN working group.

PURPOSE: To analyse the clinical characteristics of adult patients with pars planitis (PP-IU), non-pars planitis (NPP-IU) and multiple sclerosis-associated intermediate uveitis (MS-IU) and distinguish between groups. METHODS: Seventy-three adult patients with intermediate uveitis (IU) reviewed retrospectively and divided as PP-IU, NPP-IU and MS-IU according to 'The standardization of uveitis nomenclature working group classification criteria.' Demographic and clinical characteristics, OCT and fluorescein angiography (FA) findings, complications and treatments were recorded. RESULTS: A total of 134 eyes of 73 patients were included, and 42 of the patients were classified as PP-IU, 12 as NPP-IU, and 19 as MS-IU. If a patient presenting with blurred vision, or tent-shaped vitreous band/snowballs/snowbank on examination, or vascular leakage on FA and accompanying neurological symptoms, the frequency of demyelinating plaque detection on cranial MRI and the risk of MS-IU increased. Mean BCVA was increased from 0.22 ± 0.30 logMAR to 0.19 ± 0.31 logMAR (p = 0.021). Gender, initial BCVA, snowbank formation, disc oedema and periphlebitis on examination, and disc leakage/occlusion on FA were found predictive of decreased BCVA at final visit (p < 0.05). CONCLUSIONS: The clinical features of these three groups are similar, some features that can guide the differential diagnosis. It may be recommended to periodically evaluate "suspicious" patients with MRI for MS.

Macular vessel densities by optical coherence tomography angiography in patients with intermediate uveitis.

Purpose: To evaluate the macular microvascular changes in patients with intermediate uveitis (IU) using optical coherence tomography angiography (OCT-A) and to determine the relationship of OCT-A measurements with disease duration and activity. Methods: This cross-sectional study was performed at the uvea department of a tertiary hospital. Patients with IU (8 women, 8 men; mean age: 28.80 ± 12.80 years) were included in the study. The macular thickness measurements obtained by optical coherence tomography (OCT) (Heidelberg Spectralis, Heidelberg Engineering, Heidelberg, Germany) and macular superficial and deep vessel density (VD) parameters obtained by OCT-A (Avanti RTVue-XR; Optovue, Fremont, CA, USA) were compared with healthy subjects (12 men, 22 women; mean age: 28.40 ± 8.32 years). Eyes with pathology including marked obvious edema in the macula were not included in the study. Results: The macular thickness was found to be higher in the patient group (p < 0.001). The deep and superficial VD measurements in almost all quadrants in both superficial and deep layers were significantly lower in the IU patient group (p < 0.05). Macular thickness was increased in the active state of IU (p = 0.03), however, none of the OCT-A parameters showed a significant difference between active and inactive IU patients (p > 0.05). No correlation was observed between OCT-A parameters and total IU disease duration. Conclusion: Quantitative analyses of macular vascular structures demonstrate significantly reduced VD in both superficial and deep retinal layers in IU patients. Considering the importance of the macula in visual prognosis, OCT-A can provide crucial data for the monitoring and follow-up of IU patients.

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.

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.

Intermediate Uveitis in Retinitis Pigmentosa Associated with a Novel Homozygous Splice Site Mutation in PRPF8.

The manifestation of intermediate uveitis (IU) in patients with retinitis pigmentosa (RP) is uncommon and poses diagnostic and management challenges. In this case, we describe the clinical features and management outcomes in an RP patient with a novel homozygous splice site mutation in PRPF8. A 21-year-old male presented with unilateral decrease of vision in the right eye for 1 week. Retinal dystrophy features were present in the left eye. After 2 weeks of topical steroid therapy, near-total resolution of IU was achieved and vision improved to 20/30. Signs of (RP) were present bilaterally, with the right eye more affected than the left. Genetic testing indicated a novel homozygous c. 3061-6_3061-3del mutation in the PRPF8 gene. IU in young patients with RP can be effectively treated with a short course of topical steroids, sparing the need for systemic immunosuppressives. After the improvement in IU, the right eye showed more advanced RP changes.

Classification Criteria for Multiple Sclerosis-Associated Intermediate Uveitis.

PURPOSE: The purpose of this study was to determine classification criteria for multiple sclerosis-associated intermediate uveitis. DESIGN: Machine learning of cases with multiple sclerosis-associated intermediate uveitis and 4 other intermediate uveitides. METHODS: Cases of intermediate uveitides were collected in an informatics-designed preliminary database, and a final database was constructed of cases achieving supermajority agreement on the diagnosis, using formal consensus techniques. Cases were split into a training set and a validation set. Machine learning using multinomial logistic regression was used in the training set to determine a parsimonious set of criteria that minimized the misclassification rate among the intermediate uveitides. The resulting criteria were evaluated in the validation set. RESULTS: A total of 589 cases of intermediate uveitides, including 112 cases of multiple sclerosis-associated intermediate uveitis, were evaluated by machine learning. The overall accuracy for intermediate uveitides was 99.8% in the training set and 99.3% in the validation set (95% confidence interval: 96.1-99.9). Key criteria for multiple sclerosis-associated intermediate uveitis included unilateral or bilateral intermediate uveitis and multiple sclerosis diagnosed by the McDonald criteria. Key exclusions included syphilis and sarcoidosis. The misclassification rates for multiple sclerosis-associated intermediate uveitis were 0 % in the training set and 0% in the validation set. CONCLUSIONS: The criteria for multiple sclerosis-associated intermediate uveitis had a low misclassification rate and appeared to perform sufficiently well enough for use in clinical and translational research.

Classification Criteria for Intermediate Uveitis, Non-Pars Planitis Type.

PURPOSE: To determine classification criteria for intermediate uveitis, non-pars planitis type (IU-NPP, also known as undifferentiated intermediate uveitis). DESIGN: Machine learning of cases with IU-NPP and 4 other intermediate uveitides. METHODS: Cases of intermediate uveitides were collected in an informatics-designed preliminary database, and a final database was constructed of cases achieving supermajority agreement on the diagnosis, using formal consensus techniques. Cases were split into a training set and a validation set. Machine learning using multinomial logistic regression was used on the training set to determine a parsimonious set of criteria that minimized the misclassification rate among the intermediate uveitides. The resulting criteria were evaluated on the validation set. RESULTS: Five hundred eighty-nine of cases of intermediate uveitides, including 114 cases of IU-NPP, were evaluated by machine learning. The overall accuracy for intermediate uveitides was 99.8% in the training set and 99.3% in the validation set (95% confidence interval 96.1, 99.9). Key criteria for IU-NPP included unilateral or bilateral intermediate uveitis with neither snowballs in the vitreous humor nor snowbanks on the pars plana. Other key exclusions included multiple sclerosis, sarcoidosis, and syphilis. The misclassification rates for IU-NPP were 0% in the training set and 0% in the validation set. CONCLUSIONS: The criteria for IU-NPP had a low misclassification rate and seemed to perform well enough for use in clinical and translational research.

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.

Prognosis of Ocular Tuberculosis Following Long-Term Antitubercular Therapy.

Purpose: This study presents clinical features and prognosis after long-term (12-18 months) antitubercular therapy (ATT) in patients with ocular tuberculosis (OTB) in East China, an endemic area of tuberculosis. Methods: This retrospective study reviewed data from OTB patients treated at the Eye and ENT Hospital of Fudan University from 2008 to 2018. All the patients completed a minimum follow-up of 6 months after the cessation of ATT. Results: Sixty-six patients with OTB were studied. The ocular manifestations included retinal vasculitis (51.6%), choroiditis (24.2%), panuveitis (23.2%), intermediate uveitis (7.4%), scleritis (5.3%), anterior uveitis (2.1%), and optic neuropathy (1%). Except for two patients (ATT for 6 months), all other patients (64/66, 96.97%) received ATT for at least 12 months (6 patients for 12 months, 30 patients for 15 months, and 28 patients for 18 months). Treatment in conjunction with oral corticosteroids was used in 48 patients (72.7%). The average initial best-corrected visual acuity (BCVA) was 0.8 ± 0.64 (LogMAR), which improved to 0.31 ± 0.35 (LogMAR) at the last follow-up (P < 0.05). The final BCVA was significantly associated with the initial BCVA and the duration of clinical symptoms. A complete remission of uveitis was achieved in 97% of the patients. Conclusions: This study observed a favorable prognosis with long-term ATT regimens. Patients with better baseline visual acuity and a shorter duration of clinical symptoms before diagnosis had a better prognosis.

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.