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Purpose: To review the clinical features, diagnosis, and treatment of idiopathic retinal vasculitis, aneurysms, and neuroretinitis (IRVAN) and to report a case with the use of ultra-widefield fluorescein angiography (UWFA) for confirming the precise staging of IRVAN and aid in early treatment. The patient improved after being treated with intravitreal aflibercept injection., Results: A 26-year-old female complained of progressive blurred vision OD for one week. Her BCVA was 0.6 OD and 1.0 OS. Fundus examination showed vitritis, retinal hemorrhage, and vasculitis over bilateral eyes. Fluorescein angiography (FA) with a 55 degree of view revealed aneurysmal dilations of the peripapillary arteriole, peripapillary focal leakage, venous leakage, and capillary nonperfusion area. Stage 2 IRVAN was impressed OU. Oral prednisolone was administered. After four months, she experienced decreased visual acuity OS. Optical coherence tomography (OCT) revealed subretinal and intraretinal fluid with hyperreflective material. One posterior subtenon triamcinolone and one intravitreal aflibercept injection were performed OS, and macular edema subsided. A 105-degree ultra-widefield fluorescein angiography (UWFA) showed multiple peripheral background hypofluorescence areas corresponding to capillary nonperfusion. Retinal neovascularization (NV) was found OS, which had not been revealed by the previous 55-degree FA. Stage 3 IRVAN was made OS and panretinal laser photocoagulation (PRP) was performed. Oral prednisone and cyclosporine were prescribed. Her vision improved to 1.0 OU., Conclusion: UWFA provides visualization of peripheral retinal pathology and for precise staging. It also had direct implications in the follow-up and treatment strategy.

This meta-analysis aimed to analyze retinal microvasculature features in eyes with Behçet's disease (BD) using optical coherence tomography angiography (OCTA). Electronic databases, including PubMed, Web of Science, Embase, and Cochrane Library, were comprehensively searched for published studies comparing retinal microvasculature characteristics between eyes with BD and controls. Continuous variables were calculated using the mean difference (MD) with 95% confidence interval (CI). Review Manager software (version 5.30) was used to conduct statistical analysis. A total of 13 eligible studies involving 599 eyes with BD and 622 control eyes were included in the meta-analysis. The pooled results showed that the macular whole enface superficial and deep vessel density (VD) values measured by OCTA were significantly lower in eyes with BD than in control eyes (superficial VD: MD = - 3.05, P < 0.00001; deep VD: MD = - 4.05, P = 0.0004). The foveal superficial and deep VD values were also significantly lower in the BD group than in the control group (superficial VD: MD = - 1.50, P = 0.009; deep VD: MD = - 4.25, -  = 0.03). Similarly, the analysis revealed a significant reduction in the parafoveal superficial and deep VD in eyes with BD than in control eyes (superficial VD: MD =  - 3.68, P < 0.00001; deep VD: MD = - 4.95, P = 0.0007). In addition, the superficial and deep foveal avascular zones (FAZs) were significantly larger in patients with BD than in controls (superficial FAZ: MD = 0.06, P = 0.02; deep FAZ: MD = 0.12, P = 0.03). The present meta-analysis found that macular whole enface VD, foveal VD, and parafoveal VD were lower in eyes with BD, and the FAZ was larger in patients with BD. The findings suggest that OCTA can assist clinicians in diagnosing and monitoring the status of patients with BD., (© 2022. The Author(s).)

Purpose : To report a case of acute macular neuroretinopathy (AMN) in Behçet Disease. Case : A 23-year-old male presented with a complaint for central scotoma in his right eye. He had been diagnosed with Behçet Disease 3 years ago. Best-corrected visual acuity (BCVA) was 20/20. Anterior chamber and fundus examinations were unremarkable. Optical coherence tomography revealed a paracentral area of outer nuclear layer thinning. Infrared reflectance showed a well-defined, circular, hyporeflective area. Optical coherence tomography angiography revealed an area of capillary dropout in deep retinal capillary plexus corresponding to that hyporeflective lesion. Microperimetry test showed decreased macular sensitivity on the lesion area and the loss of the macular integrity. In multifocal electroretinogram, diminished amplitudes of the central cone responses were detected nasal to fixation. Conclusion : Behçet disease is a cause of occlusive retinal vasculitis. Accompanied retinal microvascular disease may be a possible risk factor of AMN suggesting ischemic etiopathogenesis for AMN.

Purpose: To describe the vascular anatomy and intraluminal flow characteristics of segmental retinal arteritis (SRA) using structural and angiographic optical coherence tomography (OCT)., Methods: Retrospective case series of consecutive patients presenting with SRA. All patients were evaluated at presentation with fundus photography, spectral domain OCT, and OCT angiography. One patient was imaged with dense B-scan OCT angiography., Results: Three eyes of three male patients were evaluated. All examinations were consistent with reactivation of ocular toxoplasmosis with an area of active retinochoroiditis adjacent to a focal chorioretinal scar. Spectral domain OCT through areas of SRA noted on clinical examination demonstrated areas of hyperreflectivity circumscribing the affected vessel with a normoreflective lumen. Optical coherence tomography angiography and dense B-scan OCT angiography demonstrated narrowing of the intraluminal flow signal that correlated with areas of segmental hyperreflectivity on spectral domain OCT. Vascular sections proximal and distal to areas of SRA showed normal flow signal., Conclusion: Vessels with SRA demonstrated hyperreflectivity highlighting the vessel wall on spectral domain OCT. Optical coherence tomography angiography showed narrowing of the flow signal within these segments suggesting reduced lumen diameter. Coupling these finding with previous indocyanine green imaging findings in SRA, the collective data suggest the plaques are localized within the vessel wall to either the endothelium or the muscular tunica media without occlusion of the vessel lumen.

To study the correlation of OCT parameters including central subfield macular thickness (CSMT), peripapillary retinal thickness (PRT), and peripapillary retinal nerve fiber layer thickness (PNFLT) with fluorescein angiography (FA) in evaluation of inflammatory activity in Behcet's retinal vasculitis. In this case-series, concurrent FA and OCT were performed. A scoring system was devised for FA. PNFLT in 3.4-mm-diameter circle as well as PRT in doughnut shaped regions between the 1-mm- and 2.2-mm-diameter and between the 2.2-mm- and 3.45-mm-diameter circles was measured. The correlation of FA and OCT parameters was analyzed. A total of 105 sets of FA from 28 eyes (15 patients) were reviewed. Four (26.6%) were female and mean age was 31.6 ± 8.49 years. Each micron increase in CSMT, PRT2.2, PRT3.45, and PNFLT, caused a rise of 0.018 (95% CI 0.008-0.027, P < 0.001, r = 0.413), 0.053 (95% CI 0.035-0.070, P < 0.001, r = 0.443), 0.086 (95% CI 0.065-0.108, P < 0.001, r = 0.707), and 0.185 (95% CI 0.152-to 0.218, P < 0.001, r = 0.850) unit in FA score, respectively. Parameters having significant correlation with angiographic inflammatory activity, were CSMT, PRT2.2, PRT3.45 and RNFLT. Those with the strongest correlation, PRT3.45 and PNFLT, may be considered as quantitative non-invasive alternatives to FA for monitoring Behcet's retinal vasculitis., (© 2021. The Author(s).)

Retinal Vasculopathy with Cerebral Leukoencephalopathy and Systemic manifestations (RVCL-S) is a small vessel disease caused by TREX1 mutations. RVCL-S is characterized by retinal vasculopathy and brain white matter lesions with and without contrast enhancement. We aimed to investigate cerebrovascular reactivity (CVR) in RVCL-S. In this cross-sectional observational study, 21 RVCL-S patients, 23 mutation-negative family members, and 31 healthy unrelated controls were included. CVR to a hypercapnic challenge was measured using dual-echo arterial spin labeling magnetic resonance imaging. Stratified analyses based on age were performed. We found that CVR was decreased in gray and white matter of RVCL-S patients compared with family members and healthy controls (ANCOVA; P  < 0.05 for all comparisons). This was most noticeable in RVCL-S patients aged ≥40 years (ANCOVA, P  < 0.05 for all comparisons). In RVCL-S patients aged < 40 years, only CVR in white matter was lower when compared to healthy controls ( P  < 0.05). Gray matter CVR was associated with white matter lesion volume in RVCL-S patients ( r  = -0.527, P  = 0.01). In conclusion, impaired cerebrovascular reactivity may play an important role in the pathophysiology of RVCL-S and may be an useful early biomarker of cerebrovascular disease severity.

Purpose: To compare quantitative optical coherence tomography angiography (OCT-A) measurements of the parafoveal microvasculature in retinal capillary plexuses among Behҫet uveitis (BU) patients, non-ocular Behҫet's disease (NOBD) patients, and healthy volunteers (HVs)., Methods: Sixty-eight subjects were enrolled in this prospective observational cross-sectional study. OCT-A imaging was performed using the Heidelberg Engineering Spectralis OCT. A custom algorithm was developed to calculate the vessel density (VD) in three retinal vascular layers: deep capillary plexus, intermediate capillary plexus, and superficial vascular plexus. The foveal avascular zone (FAZ) and acircularity index were calculated for the whole retinal vascular complex., Results: We analyzed one eye from 21 BU patients (age, 51 ± 10 years), 23 NOBD patients (age, 48 ± 14 years), and 22 HVs (age, 44 ± 13 years). One-way multivariate analysis of covariance showed a statistically significant difference in VD among the three groups when combining the layers after controlling for scan quality (P < 0.001). The VD was lowest in the BU group and highest in the HV group in all layers. The FAZ area was also statistically significant different among the groups (P < 0.005), with the largest FAZ areas in BU patients and smallest FAZ areas in the HV group. However, no statistically significant difference was found for the acircularity index., Conclusions: The parafoveal microvasculature is affected not only in BU patients but also in NOBD patients. Most deviations in the retinal microcirculation in Behҫet patients were found in the deeper layers of the retina by using the quantitative VD measurement.

Purpose : To monitor perivascular sheathing during the course of retinal vasculitis by flood illumination adaptive optics ophthalmoscopy (AOO). Methods : Perivenous sheathing and venous diameters were quantitatively analyzed by semi-automatic segmentation of AOO images in 12 eyes of treatment-naive patients with retinal vasculitis. Results : The width of venous sheathing ranged from 45 to 225 µm (mean 101.0 µm ± 54.3). In 10 cases, the underlying vein showed focal narrowing (mean ± SD 14% ± 10). Focal narrowing of arteries was also present in one eye. At presentation, width of sheathing and vessel diameters were not correlated with fluorescein leakage. During follow-up, 5 eyes showed an increase in vein diameter or resolution of narrowing and in 10 eyes a thinning of vascular sheathing was observed ( p = .003). Conclusions : Perivenous sheathing may be quantitatively analyzed and monitored by AOO. AOO may therefore contribute to monitor vascular sheathing during posterior uveitis.

Purpose: To describe a case of retinal vasculitis associated with ipilimumab., Methods: Case report., Results: A 49-year-old Caucasian man presented with anterior uveitis of the left eye. He had a history of chronic myeloid leukemia undergoing ipilimumab therapy. He was treated with topical corticosteroids and achieved inactive inflammation. Ipilimumab was stopped by his oncologist since he manifested other immune-related adverse events (irAEs). Five months later, his visual acuity decreased in his left eye and optical coherence tomography demonstrated new cystoid macular edema. Fluorescein angiography revealed diffuse capillary leakage of the left eye. He received oral prednisone with improvement of both macular edema and vascular leakage, however, both remained persistent. He received an intravitreal injection of a 0.7 mg dexamethasone implant in the left eye with subsequent resolution of macular edema and vasculitis., Conclusions: The occurrence of retinal vasculitis with ipilimumab expands the spectrum of ocular irAEs related to immune checkpoint inhibitors.

Dear Editor, A 17-year-old boy, diagnosed with Systemic Lupus Erythematosus (SLE), presented to ophthalmology department with gradual painless diminution of vision in both eyes (right more than left). He had already received 6 pulses of cyclophosphamide and steroids at monthly intervals one year back for diffuse alveolar hemorrhage (DAH) and was on maintenance oral 40 mg prednisolone and 3 grams mycophenolate mofetil (MMF). There was no history of oliguria, skin rash, joint pain, oral ulcers, photosensitivity or any neurological deficit in this presentation. There was no proteinuria, hematuria or worsening of renal function.

Background/aims: To investigate the utility of using montaged optical coherence tomography (OCT) thickness maps to monitor perivascular thickness as a marker of vasculitic activity in patients with large-vessel retinal vasculitis., Methods: This is a retrospective cohort study of 22 eyes of 11 patients with a history of retinal vasculitis associated with birdshot chorioretinopathy (BCR). Patients had serial spectral domain 6×6 mm cube OCT scans centred on the fovea, optic nerve and proximal branches of the superior and inferior retinal vessels. OCT thickness change maps for each respective region were analysed. Changes in perivascular thickness were confirmed by assessing vasculitic activity on fluorescein angiography (FA), when clinically indicated., Results: In three patients, montaged OCT scans were acquired at diagnosis and serially through initial treatment. In all three patients, montaged OCT demonstrated reduced perivascular thickening with oral prednisone treatment, which was confirmed by FA showing reduced vascular leakage in both eyes. Eight patients had serial montaged OCT scans after diagnosis and initial treatment of BCR. Four of these patients showed fluctuations in perivascular thickness during flares and treatment that were confirmed by either increased or decreased vascular leakage on FA. The other four patients remained quiet on their immunosuppressive treatment regimens, and no changes in perivascular thickness were detected., Conclusions: Evaluating large-vessel perivascular thickness on OCT scans may be a useful method for non-invasively monitoring posterior pole large-vessel retinal vasculitis., Competing Interests: Competing interests: None declared., (© Article author(s) (or their employer(s) unless otherwise stated in the text of the article) 2018. All rights reserved. No commercial use is permitted unless otherwise expressly granted.)

Purpose: The purpose of the study was to describe macular changes in treatment-naïve eyes with Eales disease using spectral domain optical coherence tomography (SD-OCT)., Methods: A cross-sectional study was done on 79 eyes of 66 patients with Eales disease. Best-corrected visual acuity (BCVA), slit-lamp biomicroscopy (SLB), indirect ophthalmoscopy, fundus fluorescein angiography (FFA), and quantitative (central macular thickness [CMT]) and qualitative analysis on SD-OCT were performed., Results: Forty-six (58.2%) eyes had macular involvement as assessed with SD-OCT, while in 33 (41.8%) eyes, macula was not affected. Macular edema was the most common feature when macula was affected followed by epiretinal membrane. Mean CMT was higher (315.3 ± 102.3 μm) in eyes with macular involvement than those without it (243.8 ± 19.3 μm). Eyes with active vasculitis involving larger vessels and neovascularization had greater chance of macular involvement. SLB and FFA alone missed 28.3% and 50% eyes with macular abnormalities on SD-OCT, respectively., Conclusion: While the clinical description of Eales disease points mainly to a peripheral location, macular involvement can be commonly picked up when SD-OCT is used. Macular involvement when present is associated with a poorer BCVA., Competing Interests: There are no conflicts of interest.

Idiopathic retinal vasculitis, aneurysms and neuroretinitis (IRVAN) syndrome is a rare entity of unknown etiology for which many hypotheses have been proposed with inflammation being the most commonly accepted hypothesis. We report cases of a 9 year old girl and a 22 year old male patient with diagnosis of IRVAN syndrome. The conclusions drawn from the clinical examination and multimodal imaging including optical coherence tomography angiography of the patients are discussed. Our conclusions and interpretation point towards IRVAN being a developmental vascular anomaly rather than a consequence of inflammation. It is thus proposed to be renamed as Idiopathic retinal arteriolar aneurysm syndrome (IRAA)., (Copyright © 2017 Elsevier Ltd. All rights reserved.)

Competing Interests: Competing interests: None declared.

The term retinal vasculitis (RV) encompasses a heterogeneous group of sight-threatening conditions that are included in the intraocular inflammatory diseases that affect the posterior segment of the eye. Based on the nature of the inflammatory process, RV are classified into predominantly inflammatory or ischaemic (occlusive RV). The diagnosis is clinical and the aetiology can be infectious or non-infectious (immune-mediated). RV can be an isolated ocular syndrome or be associated with a systemic disease, of which they can represent the first manifestation. New retinal imaging techniques such as ultra-wide field fluorescein angiography and optical coherence tomography angiography will help us classify the RV and aid the diagnostic process, which can be challenging and require a multidisciplinary approach. Therefore, clinical knowledge of RV is essential for prompt diagnosis and to establish the appropriate treatment., (Copyright © 2017 Elsevier España, S.L.U. All rights reserved.)

Retinal vasculitis presents with inflammation involving the retinal vasculature as an isolated disease or in combination with other ocular or systemic conditions. This entity may be associated with a wide variety of clinical manifestations such as vascular sheathing, cotton-wool spots, retinal ischemia, and neovascularization. Often, retinal vasculitis and its complications lead to diagnostic challenges in identifying the exact etiology of the inflammation. Ancillary investigations such as fundus photography, fluorescein angiography, and more recently, adaptive optics imaging and optical coherence tomography angiography, may provide valuable information that help in establishing the exact diagnosis and initiation of appropriate therapy. In the index review, multimodal imaging features of retinal vasculitis are described. In addition, detection of vascular inflammation using novel noncontact imaging techniques has been highlighted.

Introduction Tolosa-Hunt syndrome (THS) is one of the most common 'benign' causes of painful ophthalmoplegia. Diagnosis is based on clinical and imaging findings and the exclusion of other causes because there is no specific biomarker for the syndrome. Eales disease, an idiopathic inflammatory venous disease that primarily affects the eye, can also affect the central (as stroke or myelitis) and peripheral nervous system. Case report We report the case of a 32-year-old woman with a subacute left ophthalmoplegia and evidence of a gadolinium-enhanced lesion suggesting an inflammatory granuloma that resolved within 48 hours after treatment with steroids. A diagnosis of THS was considered at this time. On a follow-up ophthalmological examination, a diagnosis of Eales disease with involvement of the left eye was made. The patient was treated successfully. Conclusion Eales disease could be a cause of painful ophthalmoplegia and may mimic THS. Long-term follow-up of patients diagnosed with THS may be necessary to exclude other diagnoses.

Purpose: To assess the utility of ultra-wide field (UWF) versus conventional fundus imaging in the management of tubercular (TB) posterior uveitis., Methods: Twenty-two consecutive patients (33 eyes) diagnosed with TB posterior uveitis in a tertiary care center who underwent UWF fundus photography and fluorescein angiography (FA) between July 2014 and March 2015 were included. Complete clinical and imaging records of the patients were retrospectively reviewed. A circle simulating the central 75-degree field was drawn on UWF pseudocolor and fluorescein angiography images. Findings within the circle were compared with the information yielded by the complete image and its impact on patient management was noted., Results: The clinical manifestations of posterior tubercular uveitis included retinal vasculitis (17 eyes), multifocal serpiginoid choroiditis (13 eyes), choroidal granulomas (2 eyes) and intermediate uveitis (1 eye). UWF imaging revealed additional capillary non-perfusion areas, neovascularization, active vasculitis, and peripheral choroiditis lesions in 30/33 eyes (90.9%), which influenced treatment decision in 15 eyes (45.5%)., Conclusions: UWF imaging is useful in the detection of peripheral pathologies in tubercular posterior uveitis that may influence management decisions, such as addition of immunosuppressive therapy or scatter laser photocoagulation.

Case Report: We present the case of a 69-year-old woman with unilateral retinal vasculitis. Investigations showed asthma, rhinosinusitis, nasal polyposis, peripheral blood eosinophilia, increased sedimentation rate, proteinuria, and antiphospholipid antibodies. Anti-neutrophil cytoplasmic antibodies (ANCA) were negative., Discussion: Although her anti-neutrophil cytoplasmatic antibody (ANCA) status was negative, taking into account the other clinical and laboratory features, retinal vasculitis was thought to be an ocular manifestation of Churg-Strauss syndrome. Treatment was started with high-dose corticosteroids and anticoagulant therapy., (Copyright © 2016 Sociedad Española de Oftalmología. Publicado por Elsevier España, S.L.U. All rights reserved.)

Frosted branch angiitis is a special form of retinal vasculitis, which has only rarely been reported in the literature. Although the majority of cases of frosted branch angiitis are idiopathic, a systematic clinical work-up should be done in order to exclude other causes, such as infectious and autoimmune diseases. The aim of this study was to correlate the clinical findings with the images obtained during follow-up by spectral domain optical coherence tomography (OCT).

Case Report: The case is described of a patient who had a sudden loss of vision in her right eye after glaucoma surgery. A diagnosis of retinal toxicity due to tobramycin (an aminoglycoside) was reached, which was characterised by retinal whitening with a red cherry stain, macular oedema, and vasculitis that progressed to papillary and macular atrophy with arteriolar sclerosis. Given the severity of symptoms an early attempt was made with megadoses of steroids and an intravitreal dexamethasone implant (Ozurdex®, Allergan S.A.), without response., Discussion: Aminoglycoside toxicity is a rare, idiosyncratic, very serious complication for which there is no effective treatment., (Copyright © 2016 Sociedad Española de Oftalmología. Published by Elsevier España, S.L.U. All rights reserved.)

Purpose: Sudden retinal blindness is a common complication of temporal arteritis (TA). Another common cause is embolic occlusion of the central retinal artery (CRA). The aim of this prospective study was to examine the diagnostic value of hyperechoic material in the CRA for the exclusion of vasculitis as a cause. The authors used orbital color-coded sonography (OCCS) for the detection of hyperechoic material., Materials and Methods: 24 patients with sudden vision loss were included in the study after the exclusion of other causes (e. g. vitreous bleeding, retinal detachment). Parallel to routine diagnostic workup, OCCS was performed in all patients., Results: 7 patients with a diagnosis of TA presented with different degrees of hypoperfusion in the CRA without hyperechoic material (referred to as "spot sign") detected by OCCS. Diagnostic workup in the remaining 17 patients revealed other causes of sudden vision loss, such as central retinal artery occlusion (CRAO) (12), anterior ischemic optic neuropathy (AION) (2), upstream vascular stenosis or occlusion (2) and delayed reperfusion of the CRA (1). The hyperechoic "spot sign" was visible in 10 of 12 patients (83 %) with embolic CRAO. The detection of embolic CRAO using the "spot sign" had a sensitivity of 83 % and a specificity of 100 %. The missing "spot sign" in patients with TA was a highly specific finding (p-value 0.01)., Conclusion: The detection of the "spot sign" specifically minimizes the probability of TA as a reason for sudden blindness., (© Georg Thieme Verlag KG Stuttgart · New York.)

Tuberculosis commonly affects the eye by causing neovascularization, peripheral vasculitis, and choroidal tubercles. The authors describe a 28-year-old man with systemic tuberculosis who presented with a retinal vascular tumor, peripheral retinal vasculitis, retinal neovascularization, and vitreous hemorrhage causing acute vision loss. He was successfully treated with systemic anti-tuberculosis medications, retinal photocoagulation, and focal ablative diode laser to the tumor. Ophthalmologists should consider performing a purified protein derivative test and a chest x-ray for any patient with a history suspicious for tuberculosis who presents with a vascular tumor.

Eales' disease is an idiopathic occlusive vasculopathy of the retina, which is characterized by extensive peripheral non-perfusion, perivascular sheathing, and neovascularization. It is associated with recurrent vitreous hemorrhages. Both eyes are affected consecutively in 80% to 90% of the patients. In spite of the multiple theories that have been proposed, it continues to have unknown origin and its diagnosis relies on exclusion of other causes of retinal vasculopathy. In some occasions, these patients develop complications of the central nervous system, above all brain infarcts. We present the case of a 38 year old woman with Eales' disease who developed bilateral brain infarcts associated with occlusion or stenosis of the middle cerebral arteries. The cerebral angiography showed beading of the Silvian arteries, suggestive of an underlying inflammatory disorder. Early corticosteroid therapy could avoid contralateral retinal involvement and neurological complications. The patient also presented delayed jaw closing dystonia secondary to basal ganglia infarct which followed a benign course with spontaneous resolution in a few days.

Simultaneous occurrence of frosted branch angiitis and immune-mediated rapidly progressive glomerulonephritis is reported. The two diseases possibly share a common immune mechanism. Patients of frosted branch angiitis should undergo complete systemic evaluation including renal function tests even if the patient is systemically asymptomatic.