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Intermediate uveitis is a relatively common form of intraocular inflammation that can be seen at any age. The vitreous is the primary site of inflammation; and the presence of snowballs or snowbank defines its subtype, pars planitis. Since it is a clinical diagnosis, it is important to recognize the typical clinical features and associated ocular complications. Recent advances in ocular imaging have made significant contributions to the clinical appraisal and studies of intermediate uveitis. This narrative review includes clinical signs, ocular complications, and ocular imaging of intermediate uveitis and pars planitis. [ABSTRACT FROM AUTHOR]

We present a 20-year-old woman who was diagnosed with subacute sclerosing panencephalitis (SSPE) 20 months after presenting with unilateral retinitis. At presentation, the patient had two inferotemporal macular lesions in her left eye. Corresponding to these areas, optical coherence tomography (OCT) showed hyporeflective spaces with loss of nearly all of the retinal layers. OCT-angiography (OCTA) demonstrated some flow deficit areas with a reduction in the vessel density. Her serum measles antibody titre was high (IgG >5000.0 mIU/ml). Twenty months later the macular lesions had diminished in size, and there was some focal retinal thinning with interruption of the ellipsoid zone. OCTA showed that the flow deficit areas were diminished in size together with the relatively improved perfusion density. Neurological examination disclosed myoclonic jerks. Neuropsychological assessment demonstrated impaired executive function, attention, and narrowed lexical fluency. Measles IgG antibody was high in the cerebrospinal fluid (>230.0 U/ml). Brain magnetic resonance imaging demonstrated bilateral, non-specific, small foci of T2 hyperintensity in the frontoparietal subcortical white matter and centrum semiovale. The present case is the first where OCTA findings of SSPE-related retinal lesions have been described. [ABSTRACT FROM AUTHOR]

Background: The global and precise follow-up of uveitis has become possible with the availability of dual fluorescein (FA) and indocyanine green angiography (ICGA) since the mid-1990s. Progressively, additional non-invasive imaging methods have emerged, bringing value-added precision to the imaging appraisal of uveitis, including, among others, optical coherence tomography (OCT), enhanced-depth imaging OCT (EDI-OCT) and blue light fundus autofluorescence (BAF). More recently, another complementary imaging method, OCT-angiography (OCT-A), further allowed retinal and choroidal circulation to be imaged without the need for dye injection. Purpose: The purpose of this review was aimed at examining the evidence in published reports indicating whether OCT-A could possibly replace dye angiographic methods, as well as the real practical impact of OCT-A. Methods: A literature search in the PubMed database was performed using the terms OCT-angiography and uveitis, OCTA and uveitis and OCT-A and uveitis. Case reports were excluded. Articles were classified into technical reports, research reports and reviews. Articles in the two latter categories were analyzed in a more detailed, individual fashion. Special attention was paid to whether there were arguments in favor of an exclusive rather than complementary use of OCT-A. Furthermore, a synthesis of the main practical applications of OCT-A in the management of uveitis was attempted. Results: Between 2016 (the year of the first articles) and 2022, 144 articles containing the search terms were identified. After excluding case report articles, 114 articles were retained: 4 in 2016, 17 in 2017, 14 in 2018, 21 in 2019, 14 in 2020, 18 in 2021 and 26 in 2022. Seven articles contained technical information or consensus-based terminology. Ninety-two articles could be considered as clinical research articles. Of those, only two hinted in their conclusions that OCT-A could hypothetically replace dye methods. The terms mostly used to qualify the contribution of the articles in this group were "complementary to dye methods", "adjunct", "supplementing" and other similar terms. Fifteen articles were reviews, none of which hinted that OCT-A could replace dye methods. The situations where OCT-A represented a significant practical contribution to the practical appraisal of uveitis were identified. Conclusion: To date, no evidence was found in the literature that OCT-A can replace the classic dye methods; however, it can complement them. Promoting the possibility that non-invasive OCT-A can substitute the invasive dye methods is deleterious, giving the elusive impression that dye methods are no longer inevitable for evaluating uveitis patients. Nevertheless, OCT-A is a precious tool in uveitis research. [ABSTRACT FROM AUTHOR]

To report the results of interferon (IFN) α-2a treatment in patients with cystoid macular edema (CME) secondary to acute retinal necrosis (ARN). We reviewed the records of seven patients (eight eyes) who received IFNα-2a for post-ARN CME. The initial dose of IFNα-2a was 3 MIU/day and it could be tapered down to 3 MIU twice a week. Efficacy was assessed by central macular thickness (CMT) on spectral-domain optical coherence tomography and visual acuity. Age range of seven patients (four men, three women) was 36–74 years. Mean CMT decreased from 477.9 ± 167.5 μm to 367.3 ± 120.5 μm at first week, and vision improved up to five lines in five eyes. CME relapsed after cessation of IFNα-2a in all and improved following reinstitution of treatment. Treatment was discontinued in one patient because of depression. Three patients electively discontinued treatment due to poor tolerability or lack of functional improvement. IFNα-2a is an effective therapeutic option for post-ARN CME, though side effects such as fatigue, elevated liver enzymes, neutropenia, and depression may limit tolerability. Lower initial doses may be a better tolerated. [ABSTRACT FROM AUTHOR]

Purpose: Optical coherence tomography angiography (OCT-A) has been applied to uveitis and intraocular inflammation since its availability after 2014. The imaging of retinal and choroidal vascularization without the use of dyes was a major development and represented a potentially valuable tool in ocular research. In addition to such use, OCT-A is often put forward as being able to potentially replace invasive methods needing dye injection, such as fluorescein angiography (FA) and indocyanine green angiography (ICGA). The aim of this review was to establish whether OCT-A was sufficiently useful in everyday routine clinical practice to monitor disease evolution and to perform treatment adjustments to the extent that it could reliably replace the standard dye methods. Methods: Selective literature review and analysis of own data and experience. Results: OCT-A is a technologically high-grade imaging modality allowing to analyze retinal circulation in inflammatory diseases of the posterior pole with a high sensitivity useful for research purposes. However, there is no evidence that it reaches equal effectiveness in the routine management of posterior uveitis involving the retina. OCT-A is unable to show leakage. In choriocapillaritis involving pre-capillary vessels, it shows capillary drop-out but does not seem to have an advantage over ICGA except that it can be repeated easily, not being invasive, and so allows a closer follow-up. It is, however, less useful in end-choriocapillary non-perfusion, such as in MEWDS. For choroidal stromal inflammation, OCT-A is ill-suited as it only shows inconsistent secondary circulatory changes produced by choroidal foci. OCT-A seems to be useful in the diagnosis and follow-up of inflammatory chorioneovascularisation (iCNV), although dye exams are more precise in showing the activity of the iCNV. Conclusion: In summary, OCT-A is a very sensitive modality for the retinal circulation in uveitis for research purposes; it is sometimes useful for close follow of choriocapillary drop-out but not in end-capillary non-perfusion. Its use for monitoring purposes in stromal choroiditis, however, is questionable. Its claim to possibly replace classical angiographic work-up for the practical management of posterior uveitis is largely overrated. [ABSTRACT FROM AUTHOR]

Purpose: To provide an overview of the role of multimodality approach to the diagnosis and assessment of uveitic macular edema (UME). Methods: Review of literature. Results: Optical coherence tomography (OCT) has become the gold standard in the detection, quantification, monitoring of treatment, and determination of prognosis in UME. Fluorescein angiography provides essential information on blood-retinal barrier disruption as a result of intraocular inflammation and allows detection of associated retinal ischemia and neovascularization. Dye-less OCT angiography can reveal the presence of various qualitative and quantitative retinal microvascular changes mostly involving the deep capillary plexus. Reading acuity and retinal sensitivity assessed by microperimetry represent sensitive measures for visual dysfunction in UME and may supplement distance visual acuity in the follow-up of patients with UME. Conclusion: Conventional and new imaging modalities and ancillary tests complement each other in the diagnosis, assessment, and monitoring of patients with UME. [ABSTRACT FROM AUTHOR]

Uveitis is one of the fields in ophthalmology where a tremendous evolution took place in the past 25 years. Not only did we gain access to more efficient, more targeted, and better tolerated therapies, but also in parallel precise and quantitative measurement methods developed allowing the clinician to evaluate these therapies and adjust therapeutic intervention with a high degree of precision. Objective and quantitative measurement of the global level of intraocular inflammation became possible for most inflammatory diseases with direct or spill-over anterior chamber inflammation, thanks to laser flare photometry. The amount of retinal inflammation could be quantified by using fluorescein angiography to score retinal angiographic signs. Indocyanine green angiography gave imaging insight into the hitherto inaccessible choroidal compartment, rendering possible the quantification of choroiditis by scoring indocyanine green angiographic signs. Optical coherence tomography has enabled measurement and objective monitoring of retinal and choroidal thickness. This multimodal quantitative appraisal of intraocular inflammation represents an exquisite security in monitoring uveitis. What is enigmatic, however, is the slow pace with which these improvements are integrated in some areas. What is even more difficult to understand is the fact that clinical trials to assess new therapeutic agents still mostly rely on subjective parameters such as clinical evaluation of vitreous haze as a main endpoint; whereas a whole array of precise, quantitative, and objective modalities are available for the design of clinical studies. The scope of this work was to review the quantitative investigations that improved the management of uveitis in the past 2-3 decades. [ABSTRACT FROM AUTHOR]

Purpose: To report the efficacy and safety of interferon (IFN) α-2a in patients with cystoid macular edema (CME) associated with presumed ocular tuberculosis (TB).Methods: We reviewed the clinical records of 5 patients with presumed ocular TB who had been treated with IFN α-2a for recurrent CME during or after completion of anti-tubercular therapy. IFN α-2a was administered at an initial dose of 3 million IU per day and then tapered after the initial response. Treatment efficacy was assessed by central macular thickness (CMT) measurement using spectral-domain optical coherence tomography and visual acuity.Results: Three patients were men, and 2 were women. Patients were aged between 38 and 66 years. Mean CMT was 483 ± 178.6 μm at baseline, 302.3 ± 56 μm at 1 week, 312.3 ± 49.5 μm at 1 month, and 286.2 ± 31.9 μm at 3 months. Mean LogMAR visual acuity was 0.6 ± 0.4 at baseline, 0.4 ± 0.3 μm at 1 week, 0.3 ± 0.3 at 1 month, and 0.3 ± 0.3 at 3 months. The treatment was interrupted for 10 days because of neutropenia after 2 weeks in 1 patient and discontinued in another after 10 days because of intolerance. Total treatment duration was 3-24 months in the remaining 4 patients.Conclusion: The present small case series suggests that IFN α-2a may be an effective and safe therapeutic option for CME that is associated with presumed ocular TB. [ABSTRACT FROM AUTHOR]

Behçet disease is a chronic relapsing multisystem inflammatory disorder. Ocular involvement is characterized by a bilateral recurrent non-granulomatous panuveitis and retinal vasculitis. Posterior segment findings vary during the course of the disease, in parallel with the relapsing and remitting intraocular inflammation. Structural alterations occur with increased disease duration. Fluorescein angiography is the gold standard in revealing the extent and severity as well as the leaky and/or occlusive nature of retinal vasculitis. Multimodal imaging using color fundus photography, fluorescein angiography, and optical coherence tomography is essential in visualizing diagnostic features, detecting structural changes, and monitoring disease activity and response to treatment in patients with Behçet uveitis. [ABSTRACT FROM PUBLISHER]

Purpose: To describe 3 representative cases of autoimmune retinopathy (AIR). Methods: Clinical records of patients with a diagnosis of AIR were analyzed for demographic data, clinical findings, ancillary and laboratory tests, and treatment employed. Results: Three female patients diagnosed with AIR had bilateral reduction of electroretinogram amplitudes and elevation of visual field threshold within the central 30 degrees of the visual field that was disproportionately more severe than the clinical findings of retinal degeneration. The diagnoses were cancer-associated retinopathy, non-neoplastic AIR, and hereditary retinal dystrophy with secondary inflammation. Optic nerve involvement was also present in all cases. The patient with non-neoplastic AIR was successfully treated with systemic corticosteroids and immunomodulatory agents. Conclusion: High index of suspicion is essential for an early diagnosis of AIR. Visual function and electrophysiological tests should be included in the initial workup of patients who present with suggestive clinical signs and symptoms of AIR. [ABSTRACT FROM AUTHOR]

Juvenile idiopathic arthritis-associated uveitis (JIAU) accounts for a sizable proportion of uveitis cases in children and is an important cause of ocular morbidity in uveitis patients in this age group. The authors present the results of a survey conducted to obtain a better understanding of the current views and practices of ophthalmologists involved in the care of children with JIAU. A detailed questionnaire consisting of 54 questions addressing epidemiology, diagnosis, and therapy of JIAU was distributed to 67 uveitis specialists. The responses from 37 completed questionnaires were tabulated for this report. While the experts often agreed on aspects of the epidemiologic and clinical features of JIAU and its complications, considerable diversity of responses was noted-particularly with regard to practice patterns. Regarding diagnostics and disease monitoring, all experts favored ANA testing, whereas two-thirds also suggested HLA-B27 typing. Laser flare photometry was available to and routinely used by almost one-third of the experts. Optical coherence tomography (OCT) was used by more than half. The survey revealed an overall consensus on therapeutic strategies, including the use of both conventional immunosuppressive and biologic agents. Methotrexate was the initial choice for immunosuppression by most respondents. Most would add an anti-TNF-alpha agent following failure of traditional immunosuppressive therapy, and adalimumab was favored by almost half of the experts. Questions addressing the management of individual situations, such as the treatment of macular edema and perioperative management, revealed considerable differences in therapeutic approaches. The results of this survey support the development of international guidelines for the management of JIAU. [ABSTRACT FROM AUTHOR]

The article evaluates the effectiveness of different imaging techniques in diagnosis and management of Behcet disease. The analysis reveals that fundus photography, fluorescein Angiography (FA) and optical coherence tomography (OCT) are the best methods for evaluating and monitoring Behcet disease patients. The article also describes the images obtained for Behcet disease.

The choroid was poorly accessible to imaging investigation until the last decade of the last century. With the availability of more precise imaging methods such as indocyanine green angiography (ICGA) and, later, optical coherence tomography (OCT), enhanced depth OCT (EDI-OCT), and OCT angiography (OCTA), appraisal of choroidal inflammation has substantially gained in accuracy. This allowed to precisely determine which structures were touched in the different non-infectious choroiditis entities and made it possible to classify this group of diseases, ICGA signs, mainly hypofluorescent lesions, were identified and described. Previous publications have divided angiographic findings into two main sets of signs: (1) irregular "geographic" hypofluorescent areas corresponding to choriocapillaris non-perfusion and (2) round more regular, hypofluorescent dark dots more evenly distributed in the fundus corresponding to more deep choroidal stromal foci. These distinct findings allowed to subdivide and classify choroiditis into choriocapillaritis and stromal choroiditis. Additional signs were identified from EDI-OCT and OCTA examination supporting the classification of choroiditis into choriocapillaritis and stromal choroiditis. Results: Diseases involving principally the choriocapillaris included Multiple Evanescent White Dot Syndrome (MEWDS), Acute Posterior Multifocal Placoid Pigment Epitheliopathy (APMPPE), Idiopathic Multifocal Choroiditis (MFC), and Serpiginous Choroiditis (SC) as well as mixed forms. Diseases primarily involving the choroidal stroma included HLA-A29 Birdshot Retinochoroiditis (BRC), Vogt-Koyanagi-Harada disease (VKH), Sympathetic Ophthalmia (SO), and Sarcoidosis chorioretinitis (SARC). Thanks to new imaging investigations of the choroid, it is now possible to classify and understand the diverse clinicopathological mechanisms in the group of non-infectious choroiditis entities. [ABSTRACT FROM AUTHOR]

PURPOSE: To describe nonglaucomatous retinal nerve fiber layer (RNFL) defects in patients with Behcet uveitis. DESIGN: Cross-sectional study and observational case series. METHODS: We reviewed the clinical photographs of patients with Behcet uveitis (n = 259), ocular toxoplasmosis (n = 120), and multiple sclerosis (MS)-associated uveitis (n = 40) for the presence of localized RNFL defects. Behcet patients with localized RNFL defects were invited to participate in a prospective evaluation including standard automated perimetry, spectraldomain optical coherence tomography (SD OCT), and RNFL thickness analysis. Main outcome measures were the prevalence of localized RNFL defects, perimetric and SD OCT findings, and RNFL thickness analysis. RESULTS: Sixty-two patients with Behcet uveitis (24%) had localized RNFL defect(s) without any visible scar. Twenty patients (17%) with ocular toxoplasmosis had a localized RNFL defect associated with a retinochoroidal scar in all. None of the MS patients had a localized RNFL defect. Of the 19 patients (24 eyes) with RNFL defect associated with Behcet uveitis who returned for follow-up, there was a corollary thinning on SD OCT B-scan in all and a corresponding visual field defect in 83%. RNFL thickness analysis was within normal limits in 54%, but revealed thinning in the thickness profile scale in 46% and in the pie chart in 21%. CONCLUSION: In Behcet uveitis, localized RNFL defects may be caused by microvascular ischemia at the optic nerve head and/or at the posterior pole and may serve as a helpful ocular diagnostic clue and an indicator of posterior pole involvement, a risk factor for poor visual prognosis. [ABSTRACT FROM AUTHOR]