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.

Blue-Light Fundus Autofluorescence (BAF), an Essential Modality for the Evaluation of Inflammatory Diseases of the Photoreceptors: An Imaging Narrative.

Our purpose is to describe blue-light fundus autofluorescence (BAF) features of inflammatory diseases of the outer retina characterised by photoreceptor damage. BAF from patients diagnosed with secondary and primary inflammatory photoreceptor damage were retrospectively analyzed and compared to other imaging modalities including fluorescein angiography (FA), indocyanine green angiography (ICGA), and spectral domain optical coherence tomography (SD-OCT). Multiple evanescent white dot syndrome (MEWDS), idiopathic multifocal choroiditis (MFC), acute posterior multifocal placoid pigment epitheliopathy (APMPPE), serpiginous choroiditis (SC), and acute syphilitic posterior placoid chorioretinitis (ASPPC), all cases corresponding to secondary photoreceptor diseases caused by inflammatory choriocapillaris nonperfusion, were included and compared to primary photoreceptor disease entities, including acute zonal occult outer retinopathy (AZOOR) and cancer-associated retinopathy (CAR). Both groups showed increased BAFs of variable intensity. In severe cases of APMPPE and ASPPC, BAF also showed hypoautofluorescent areas. In group 1 (secondary diseases) BAF hyperautofluorescent areas were associated with colocalized ICGA hypofluorescent areas, indicating choriocapillaris nonperfusion; whereas in group 2 (primary diseases), no ICGA signs were detected. The associated colocalized areas of hypofluorescence on ICGA in the first group, which were absent in the second group, were crucial to allow the differentiation between primary (photoreceptoritis) and secondary (choriocapillaritis) photoreceptor diseases. BAF patterns in inflammatory diseases of the outer retina can give relevant information on the photoreceptor and RPE involvement, with ICGA being crucial to detect concurring choriocapillaris damage and differentiating the two pathologies.

Expanded Spectrum of Optical Coherence Tomography Findings in Patients with Ocular Toxoplasmosis.

PURPOSE: To describe the optical coherence tomography (OCT) findings of toxoplasmic retinochoroiditis at different stages of activity. METHODS: Observational case series. RESULTS: A total of 32 eyes of 31 patients were included; 43 sets of OCT were reviewed. A total of 14 lesions were classified as active, 13 as partially active, and 16 as inactive. All active lesions demonstrated increased retinal thickness and reflectivity with blurring of details of retinal layers. Choroidal granuloma was detected in eight (61.5%) and serous retinal detachment in nine (64%). In partially active lesions, sustained thickening and/or attachment of posterior hyaloid face with fine epiretinal membrane was the hallmark. Scarified lesions showed decreased retinal and choroidal thickness starting from the periphery. Characteristic signs for decreased activity of a lesion seen in majority of both partially active and inactive lesions were RPE changes and retina-RPE approximation. We called this unique feature 'hourglass configuration'. CONCLUSION: Features in OCT are helpful to specify and monitor the activity of toxoplasmic retinochoroiditis.

Efficacy of Biosimilar Adalimumab in the Treatment of Behçet's Uveitis.

PURPOSE: This study aimed to evaluate biosimilar adalimumab's efficacy and safety in patients with Behçet's uveitis in Iran. METHODS: We performed a study on patients who mostly (79.2%) had a failure on conventional treatment with the mean follow-up time of 19.24 months (95% confidence interval (CI), 16.52-21.96). All the enrolled patients were anti-tumor necrosis factor (anti-TNF) naiive. The primary endpoint was best-corrected visual acuity (BCVA) improvement, and the secondary endpoints were changes in macular thickness, vitreous haze grade, anterior chamber (AC) cell grade, prednisolone dose, and the incidence of adverse reactions. RESULTS: Forty-eight patients were enrolled in the study. After adalimumab use, visual acuity improved significantly (p-value˂.001); vitreous haze grade decreased (p-value˂.001), and AC cell grade improved (p-value = .002). Macular thickness decreased, but its change was not statistically significant (p-value = .1). Moreover, adalimumab showed a corticosteroid-sparing effect (p-value = .03). CONCLUSION: Biosimilar adalimumab (CinnoRA®) is effective and well-tolerated in Behçet's uveitis.

Catching the therapeutic window of opportunity in early initial-onset Vogt-Koyanagi-Harada uveitis can cure the disease.

PURPOSE: Vogt-Koyanagi-Harada (VKH) disease is a primary autoimmune granulomatous choroiditis that begins in the choroidal stroma. The aim of this review was to gather a body of evidence for the concept of a window of therapeutic opportunity, defined as a time interval following initial-onset disease during which adequate treatment will substantially modify the disease outcome and possibly even lead to cure, similar to what has been described for rheumatoid arthritis. METHODS: We reviewed the literature and consulted leading experts in VKH disease to determine the consensus for the notion of a therapeutic window of opportunity in VKH disease. RESULTS: We found a substantial body of evidence in the literature that a therapeutic window of opportunity exists for initial-onset acute uveitis associated with VKH disease. The disease outcome can be substantially improved if dual systemic steroidal and non-steroidal immunosuppressants are given within 2-3 weeks of the onset of initial VKH disease, avoiding evolution to chronic disease and development of "sunset glow fundus." Several studies additionally report series in which the disease could be cured, using such an approach. CONCLUSIONS: There is substantial evidence for a therapeutic window of opportunity in initial-onset acute VKH disease. Timely and adequate treatment led to substantial improvement of disease outcome and prevented chronic evolution and "sunset glow fundus," and very early treatment led to the cure after discontinuation of therapy in several series, likely due to the fact that the choroid is the sole origin of inflammation in VKH disease.

Subclinical Inflammatory Response: Accelerated versus Standard Corneal Cross-Linking.

Purpose: To compare the subclinical inflammatory response (as measured by anterior chamber flare) induced after standard (3 mW/cm2, 30 min) and accelerated (18 mW/cm2, 5 min) corneal cross-linking (CXL). Methods: In this comparative, non-randomized study, patients with progressive keratoconus who underwent standard or accelerated CXL were studied. Laser flare photometery (FM-600; Kowa, Tokyo, Japan) was used to measure anterior chamber flare preoperatively and at 1 week, 1 month, 3 months, and 6 months after the procedure. Results: Sixty eyes of 60 patients were studied; 30 eyes in each group. Mean baseline flare values were 4.15 ± 1.19 and 4.57 ± 2.17 ph/ms in standard and accelerated groups, respectively (p = 0.228).and after surgery increased in all follow-up measurements in the both groups similarly (P > 0.05). Conclusion: Both standard and accelerated CXL results in induction of a subclinical inflammatory response that persists up to 6 month. The response was similar between the two groups.

Chronic subclinical inflammation after phakic intraocular lenses implantation: Comparison between Artisan and Artiflex models.

PURPOSE: To compare chronic subclinical inflammation induced after implantation of Artisan vs. Artiflex phakic intraocular lenses (pIOLs). METHODS: This prospective, comparative, non-randomized study included consecutive patients with moderate to high myopia who underwent Artisan or Artiflex pIOL implantation with standard surgery and postoperative care. Anterior chamber flare was assessed quantitatively using laser flare photometry (LFP) at baseline, 1 week, 1 month, 3 months, 6 months, and 2 years after surgery. RESULTS: PIOLs were implanted in 72 eyes (40 patients); Artisan pIOLs in 16 eyes (Artisan group) and Artiflex pIOLs in 56 eyes (Artiflex group). The mean preoperative anterior chamber flare was 6.5 ± 2.3 (range, 4.2-9.5) photons per millisecond (ph/ms) and 4.2 ± 0.9 (range, 2.5-11.7) ph/ms in Artisan and Artiflex groups, respectively (P = 0.400). In spite of early postoperative rise, the flare value returned to preoperative levels 6 months after pIOL implantation and remained stable up to 2 years. The amount of flare was not statistically different between Artisan and Artiflex groups in any postoperative follow-up (all P > 0.05). The trend in flare changes was not different between the studied groups (ANCOVA, P = 0.815). CONCLUSION: The inflammatory response induced by implantation of either type of Artisan and Artiflex pIOLs is short-lived without statistically significant difference between the two models.

Intraocular Silicone Oil Masquerading as Terson Syndrome.

Introduction. Terson syndrome is described as intraocular hemorrhage in association with any type of intracranial hemorrhage and is associated with higher mortality rate and vision loss. Intraocular hemorrhage in Terson syndrome may be diagnosed using computed tomography but there are false positive results. Silicone oil which is widely used for internal tamponade of complicated retinal detachments has high attenuation on computed tomography and hyperintensity on T1-weighted magnetic resonance imaging that can mimic intraocular hemorrhage. This report shows that silicone oil is another origin of false positive results in interpreting CT findings for detecting Terson syndrome. Case Report. A 71-year-old diabetic woman presented with loss of consciousness. Brain computed tomography revealed right cerebellar hemorrhage and ventricular hemorrhage and hyperdensity in vitreous cavity of the left eye that was initially interpreted as vitreous hemorrhage. Terson syndrome was the initial diagnosis but ophthalmoscopic examination and brain MRI showed that the left eye had silicone oil tamponade. Conclusion. Without knowing the history of previous vitreoretinal surgery, CT scan findings of intraocular silicone oil may be interpreted as vitreous hemorrhage. In patients with concomitant intracranial hemorrhage, it can masquerade as Terson syndrome.

Adalimumab for treatment of idiopathic frosted branch angiitis: a case report.

PURPOSE: To report a case of recurrent idiopathic frosted branch angiitis (FBA) successfully treated with adalimumab. CASE REPORT: A 14-year-old otherwise healthy boy was referred to the uveitis clinic for bilateral panuveitis with diffuse retinal vascular sheathing and severe macular edema. Extensive work-up including aqueous sampling for detection of viral causes was inconclusive. Two years ago, a similar episode had been treated with oral prednisolone, however it was complicated by adverse psychiatric effects. The progressive course of the condition mandated considering other therapeutic measures; Adalimumab was chosen based on its purported efficacy for treatment of childhood uveitis and a favorable safety profile. The patient responded dramatically to a single subcutaneous injection of adalimumab without any side effect during and after injection. The therapeutic effect was rapid and relatively long-lasting. CONCLUSION: To the best of our knowledge, this is the first case of idiopathic FBA treated successfully with adalimumab without adjunctive steroid therapy.