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Systemic vasculitides are a large group of heterogeneous diseases characterized by inflammatory destruction of blood vessels targeting diverse organs and tissues including the eye. As the most vascularized layer of the eye, the choroid is expected to be affected in multiple systemic rheumatologic diseases with vascular involvement. While there are plenty of studies investigating retinal vascular involvement, choroidal vascular involvement in systemic vasculitides has not been investigated in isolation. However, choroidal manifestations including thickness changes, choroidal vasculitis and ischemia may be the earliest diagnostic features of systemic vasculitic diseases. Thus, multimodal imaging of the choroid may help early detection of choroidal involvement which may also have prognostic implications in these life-threatening diseases. This article aimed to review involvement of the choroid in systemic vasculitic diseases. [ABSTRACT FROM AUTHOR]

Background: Multiple evanescent white dot syndrome (MEWDS) is a rare inflammatory eye condition affecting the outer retina as a consequence of choriocapillaris non perfusion. The pathophysiology of MEWDS will be discussed based clinical appraisal and on multimodal imaging appraisal. Methods: Narrative review and perspective opinion. Results: Literature review results helped us to put forward (1) the specific symptomatology (decreased/blurred vision, photopsia, subjective scotomas), (2) the ill-asserted character of clinical findings (foveal granularity, white dots in fundoscopy), (3) and the crucial importance of multimodal imaging with the diagnostic triad of ICGA hypofluorescent areas, BL-FAF hyperautofluorescent areas and loss/damage of IS/OS-ellipsoid zone on SD-OCT that characterise the disease and can practically help the clinician to diagnose MEWDS. A comprehensive alternative perspective of the disease was formulated. Conclusions: The bulk of evidence that we are presenting in this review, thanks to new performing non-invasive and invasive imaging modalities, is sufficiently compelling to consider MEWDS as a primary choriocapillaritis/inflammatory choriocapillaropathy. Multimodal imaging allows the clinician to diagnose MEWDS with a high level of certainty and ensures a precise follow-up. [ABSTRACT FROM AUTHOR]

Purpose: To analyze predisposing conditions in Turkish patients with CMV retinitis and to compare HIV-positive and HIV-negative patients. Methods: We reviewed medical charts and ocular images of 41 patients with CMV retinitis diagnosed between 1996 and 2019. Results: Eleven patients (27%) had HIV infection and 30 were immunocompromised from diverse causes. Initial visual acuity, type, zone, and extent of CMV retinitis, and response to anti-CMV treatment were not significantly different between the two groups. Vitreous haze and panretinal occlusive vasculopathy were the presenting features only in non-HIV patients, seen in 34% and 16% of eyes, respectively. Although not statistically significant, recurrent CMV retinitis was more common in non-HIV patients (17.4% vs. 4.3%/eye-year) and immune recovery uveitis was more common in HIV patients (43% vs. 26%/eye-year). Visual outcomes were similar. Final visual acuity of 1 logMAR or worse was significantly associated with the recurrence of CMV retinitis (odds ratio 9.67; p = 0.01) and also with the occurrence of immune recovery uveitis (odds ratio 4.31; p = 0.058). Conclusions: Diverse immunocompromising conditions are more commonly associated with CMV retinitis than HIV infection in Turkish patients. Intraocular inflammation was more commonly associated with active retinitis in non-HIV patients and immune recovery uveitis was more common in HIV patients. [ABSTRACT FROM AUTHOR]

Background/Purpose Vogt–Koyanagi–Harada (VKH) disease is a primary autoimmune stromal choroiditis producing a spill-over panuveitis. For initial-onset VKH disease, it is increasingly thought that corticosteroid therapy is not sufficient and additional non-steroidal immunosuppressive therapy is needed. At the 11th workshop on VKH, the disease was said to be well controlled with corticosteroids alone in Japanese patients. The aim of this study was to review the literature to determine whether different levels of severity exist in different geographical areas. Methods: Literature was reviewed for studies on the evolution of initial-onset VKH disease, looking at treatment modalities and proportion of cases with chronic evolution and/or sunset-glow fundus (SGF). Results: PubMed search yielded 1249 references containing the term of Vogt–Koyanagi–Harada. Twenty references (15 from outside of Japan and 5 from Japan) contained information on the evolution of treated initial-onset disease. For the "international" group, percentage of chronic evolution after systemic corticosteroid monotherapy was 61%, and after combined steroidal and non-steroidal therapy it fell to 2% (0% in 3/4 studies). In the Japanese studies where all patients received systemic corticosteroids alone, chronic evolution was reported in 25%; however, SGF amounted to 61%. Conclusion: In the world at large, chronic evolution of initial-onset VKH disease treated with corticosteroids alone concerned two-thirds of patients. Japanese studies showed that chronic evolution was substantially less frequent, indicating possibly less severe disease in Japan. This proportion fell to almost zero when dual steroidal and non-steroidal immunosuppression was given at onset. [ABSTRACT FROM AUTHOR]

Purpose: Vogt-Koyanagi-Harada (VKH) disease is a primary autoimmune stromal choroiditis. Aim of the study was to gather a body of evidence from the literature and from experts that systemic corticosteroid combined with non-steroidal immunosuppressive therapy should become the standard of care in initial-onset VKH disease. Methods: Literature was reviewed and leading experts in VKH were consulted in different parts of the world in order to put forward a consensus attitude in the management of initial-onset VKH disease. Results: There was a substantial body of evidence in the literature that early aggressive and sustained corticosteroid and non-steroidal immunosuppressive therapy in initial-onset VKH disease allows to achieve full control of choroidal inflammation, eliminating any subclinical choroidal inflammation, and substantially reduces recurrences with improvement of anatomical and functional outcomes. This was in agreement with experts' opinion and practice. ICGA was the method of choice to monitor disease evolution. Conclusion: Since the choroidal space is easily accessible to systemic therapy and because inflammation in VKH disease is exclusively originating from the choroidal stroma, early and sustained treatment right at the onset of the disease process with dual corticosteroid and non-steroidal immunosuppressive therapy can result in full 'healing' in many cases preventing sunset glow fundus which results from depigmentation from chronic uncontrolled inflammation. [ABSTRACT FROM AUTHOR]

Laser flare photometry (LFP) is an objective and quantitative method to measure intraocular inflammation. The LFP technology was developed in Japan and has been commercially available since 1990. The aim of this work was to review the application of LFP in uveitis practice in Europe compared to Japan where the technology was born. We reviewed PubMed articles published on LFP and uveitis. Although LFP has been largely integrated in routine uveitis practice in Europe, it has been comparatively neglected in Japan and still has not received FDA approval in the USA. As LFP is the only method that provides a precise measure of intraocular inflammation, it should be used as a gold standard in uveitis centres worldwide. [ABSTRACT FROM AUTHOR]

Purpose: The purpose of this study was to investigate the safety and effectiveness of an intravitreally injected dexamethasone-containing implant (Ozurdex) in the treatment of uveitis in children. Methods: The study group included ten patients (14 eyes) aged 6.5-15 years (mean age 12 years) with intermediate or posterior uveitis who were treated with the Ozurdex implant at two tertiary medical centers between 2009 and 2014, following an insufficient response to standard uveitis therapy. All were followed for at least 6 months (mean 12.2 ± 4.9 months). Clinical data before and after treatment were collected retrospectively from the medical files. Outcome measures were best corrected visual acuity, vitreous haze, and macular thickness. Ocular complications were documented. Results: Visual acuity improved in 12 eyes (86 %) and intraocular inflammation decreased in 13 eyes (93 %) from 1 week to 3 months after the first injection. Macular edema decreased in all eyes from 1 month to 3 months after the first injection. Five patients underwent repeated injections because of an increase in macular thickness at 3-6 month follow-up. Complications included cataract progression in one patient after one injection and cataract formation in two patients after two injections, and an elevation in intraocular pressure in two patients who responded well to topical treatment. Conclusions: Both single and repeated injections of a dexamethasone-containing implant are safe and effective for the treatment of noninfectious intermediate and posterior uveitis in children. The duration of the beneficial effect may be limited. More data are required to establish the safety profile of the implant in the pediatric age group. [ABSTRACT FROM AUTHOR]

Background: Recombinant human (rh) interferon alpha2a (IFN-α2a) therapy is successfully used for the treatment of Behçet's disease (BD) uveitis refractory to conventional immunosuppressive treatment. Purpose: Our aim in this study was to investigate the frequency and clinical significance of anti-IFN-α antibodies and autoantibodies during recombinant human rhIFN-α2a therapy in patients with BD uveitis. Methods: This comparative, cross-sectional, serological screening study included 30 BD patients treated with rhIFN-α2a (Group 1), 29 BD patients treated with conventional immunosuppressive agents (Group 2), 29 BD patients who received only colchicine (Group 3), and 30 healthy subjects (Group 4). Anti-IFN-α-binding antibodies and autoantibodies, including anti-nuclear antibody, anti-thyroid peroxidase antibody, and anti-cardiolipin antibody, were measured in serum samples. Antibody seropositivity was compared between study groups. Retrospective clinical data were compared between antibody-positive and antibody-negative patients. Results: A significantly higher proportion of patients in Group 1 had anti-interferon-α (26.6 %) and autoantibody (30 %) seropositivity compared to the other groups. No correlation was found between seropositivity for anti-interferon-α and other autoantibodies. No significant difference was found in cumulative dose of IFN-α, duration of IFN-α therapy, time to first uveitis attack, or attack rate between anti-interferon-α antibody-positive and antibody-negative patients in Group 1. Uveitis attacks were observed in 22 % of autoantibody-positive and 71 % of autoantibody-negative patients in Group 1 ( p = 0.018). Conclusions: Patients with BD uveitis develop anti-IFN-α-binding antibodies and autoantibodies during treatment with rhIFN-α2a. While the clinical relevance of anti-IFN-α-binding antibodies remains unclear in this study, induction of autoimmunity was found to be associated with a tendency for better therapeutic response. [ABSTRACT FROM AUTHOR]

We report a unique case of bilateral intraocular calcification due to necrotizing cytomegalovirus (CMV) retinitis associated with congenital CMV infection. A 7-month-old boy with a history of congenital CMV infection showed bilateral intraocular calcific plaques on computed tomography (CT) and ultrasonography. We reviewed the patient's medical files for the purpose of this report. The patient had a prior medical history of hospitalization for fever and swelling in the neck at 3 months of age. Systemic findings (anemia, neutropenia, hepatosplenomegaly, and reactive lymphadenomegaly) in association with a low CD4 count, high blood CMV viral load, and positivity for urine CMV DNA by polymerase chain reaction led to the diagnosis of bone marrow suppression and congenital CMV infection. At 7 months, he developed horizontal nystagmus and bilateral leukocoria over 20 days. Cranial CT and ultrasonography revealed bilateral intraocular calcific plaques and the patient was referred to rule out retinoblastoma. Fundoscopy was consistent with bilateral hemorrhagic, necrotizing CMV retinitis. Significant resolution of the retinal infiltrations occurred 2 weeks after initiation of systemic treatment with ganciclovir. Intraocular calcification may be a sign of active CMV retinitis. To our knowledge this is the first report of bilateral intraocular calcification serving as the presenting clinical manifestation of necrotizing CMV retinitis. [ABSTRACT FROM AUTHOR]

We report a case of subacute sclerosing panencephalitis (SSPE) presenting first with optic neuritis and rapidly progressive necrotizing retinitis at the posterior pole. We reviewed the clinical, laboratory, photographic, angiographic, and histopathologic records of a patient with SSPE. A 15-year-old girl was referred after rapid loss of vision due to optic neuritis and macular necrosis in the right eye. She had a history of cardiac valve surgery, but had no systemic symptoms and extensive work-up was unrewarding. Contralateral involvement with rapidly progressive optic neuritis and macular necrotizing retinitis prompted retinochoroidal biopsy of the right eye, which revealed necrosis of inner retinal layers and perivascular lymphoplasmocytic infiltration with intact choroid and outer retina without any findings of inclusion bodies, microorganisms, or atypical cells. The diagnosis was based on histopathologic findings consistent with SSPE, and detection of elevated measles antibody titers in cerebrospinal fluid and serum. It was further confirmed by development of typical electroencephalography pattern at 6 months and neurological symptoms at 4-year follow-up. Clinicians need to be aware that optic neuritis and necrotizing retinitis at the posterior pole may be the presenting features of SSPE. [ABSTRACT FROM AUTHOR]