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Purpose: In 15 patients with idiopathic uveitis associated with retinal vasculitis, HLA DRB1 gene testing was performed to detect a possible association. 11 patients tested positive and 4 negative for the HLA DRB1 × 15 allele. The presence of the HLA DRB1 × 15 haplotype might be associated with a higher susceptibility to develop Multiple Sclerosis (MS)., Methods: In this case series, we describe the ophthalmological and neurological findings in 10 HLA DR15-positive patients and 4 HLA DR15-negative patients that had neurological workup, including Magnetic Resonance Imaging (MRI) of the brain., Results: All patients had granulomatous ocular inflammation with either panuveitis or intermediate uveitis. MRI of the brain showed white matter lesions in 13 patients (9/10 and 4/4 respectively) of which 4 patients were eventually diagnosed with MS (3/10 and 1/4 respectively)., Conclusion: Although the majority of tested patients was carrying at least one HLA DRB1-15 allele, there was no difference in ophthalmological and neurological findings in both groups.

Purpose: To assess the validity of retinal vasculitis as the preferred diagnostic term for multiple conditions., Design: Perspective., Methods: This Perspective is based on expert opinion and review of literature focused on the current nosology and pathology of retinal vasculitis. Interpretation of the subset of intraocular inflammation named "retinal vasculitis" based on fundamental knowledge of the blood-retinal barrier, the neurovascular unit, and pathological and functional responses to a variety of stimuli is provided. Correlation with multimodal imaging and known mechanisms of immunologically mediated disease are discussed., Results: A search of Medline in early 2024 for the phrase "retinal vasculitis" resulted in 2041 citations encompassing immunologic, genetic, neoplastic, infectious, drug-related, and ischemia-related disorders. Classification schemes and angiographic grading systems are descriptive and do not address pathologic mechanisms adequately, in part due to lack of histologic confirmation. Although optical coherence tomography (OCT) angiography holds promise for better imaging of retinal vascular changes, it does not reveal the key feature of leakage and only partially improves understanding of pathophysiology. Diagnosing catastrophic retinal vascular occlusion after intravitreal injections as a retinal vasculitis is the most recent example of speculative application of the term to complex and rare disorders., Conclusions: Retinal vasculitis is a diagnostic term that is over-used and imprecise. Revised nosology should limit the term to primary inflammation of the retinal vasculature itself that results in opening of the blood-retinal barrier with or without retinal vascular occlusions. Pending new histologic or mechanistic evidence, the provisional term of "retinal vascular inflammation" or "retinal vasculopathy" should be used for leakage or occlusion occurring in the context of intraocular inflammation., Competing Interests: Conflicts of interest No conflict., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Purpose: To investigate the rare manifestation of retinal vasculitis in Familial Mediterranean fever (FMF) and its correlation with specific gene mutations, particularly the MEFV gene, with a focus on the severity of phenotypes and systemic vasculitis., Methods: A case report of a 45-year-old Armenian patient with FMF history and dual mutations (M680I and M694V) was analyzed. Clinical assessments, including ocular examinations, were conducted at various stages of the disease. Treatment modalities, including prednisone, Anakinra, and Canakinumab, were administered and their effectiveness was assessed., Results: The patient presented with bilateral ocular pain and decreased vision, exhibiting acute anterior uveitis, perivascular hemorrhages resembling Roth spots, and subsequent features of persistent vascular sheathing and cotton-wool spots. Dual mutations, especially M694V, were associated with a severe phenotype and systemic vasculitis. Treatment with prednisone induced remission, and IL-1 pathway inhibition with Anakinra and Canakinumab successfully managed relapses., Conclusion: This case underscores the rarity of retinal vasculitis in FMF, particularly involving arteries, and highlights the correlation between specific gene mutations (M680I, M694V) and disease severity. The successful management with IL-1 pathway inhibitors suggests a potential therapeutic approach. Increased clinical awareness, further research, and reporting are crucial for optimizing the understanding and treatment of FMF-related ocular manifestations.

Rationale: Brolucizumab is an anti-vascular endothelial growth factor agent. Clinical trials have demonstrated excellent efficacy of brolucizumab for neovascular age-related macular degeneration in terms of both visual and anatomic outcomes. However, compared with conventional anti-vascular endothelial growth factor therapy, this new treatment has a higher incidence of complications, particularly the development of occlusive retinal vasculitis. In this case report, we describe a patient who developed occlusive retinal vasculitis following brolucizumab treatment for age-related macular degeneration, followed by scleritis 141 days later., Patient Concerns: A 67-year-old Japanese man with a diagnosis of polypoidal choroidal vasculopathy in his right eye has received 18 intravitreal injections of aflibercept in the past 4 years. Because of a decline in treatment efficacy, intravitreal brolucizumab injection (IVBr) was initiated. However, 17 days after the second IVBr, the patient developed extensive occlusive retinal vasculitis with intraocular inflammation., Diagnosis: Occlusive retinal vasculitis in the right eye was diagnosed as a complication of brolucizumab therapy., Interventions: Corticosteroid treatment was initiated., Outcomes: The occlusive retinal vasculitis resolved 121 days after the second IVBr, and corticosteroid treatment was discontinued on day 138. However, on day 158 after the second IVBr, scleritis with intraocular inflammation developed. By day 184 after the second IVBr, both the scleritis and intraocular inflammation had resolved with the resumption of topical corticosteroid treatment., Lessons: This case underscores the potential for brolucizumab-induced scleritis and emphasizes the importance of recognizing and promptly managing this complication. Furthermore, it highlights the need for long-term careful follow-up in patients who develop occlusive retinal vasculitis after brolucizumab treatment., Competing Interests: The authors have no funding and conflicts of interest to disclose., (Copyright © 2024 the Author(s). Published by Wolters Kluwer Health, Inc.)

This case report describes a case of retinal vasculitis in a patient with Rhupus, which has not been reported earlier in the literature. A 39-year-old female patient with a history of Rhupus presented with sudden vision loss in her left eye. The patient was treated earlier, for her polyarthritis, with oral corticosteroids, hydroxychloroquine, and oral methotrexate, which were discontinued after 1 year of treatment. At presentation, fundus examination of the left eye revealed mild vitritis, perivenous sheathing, and hemorrhagic periphlebitis. The patient was administered three doses of pulse corticosteroid, resulting in a reduction in retinal vasculitis and macular edema. The patient was continued on oral corticosteroids and started on oral methotrexate and hydroxychloroquine. At 6-week follow-up, the patient's vision had improved to 6/9, and fundus examination revealed resolving phlebitis and retinal hemorrhages. This case also highlights the importance of prompt diagnosis and treatment of retinal vasculitis in patients with Rhupus.

Background: Eales' disease is an idiopathic, inflammatory condition characterized by peripheral retinal phlebitis, distal non-perfusion, and neovascularization. Coats' disease, on the other hand, is an idiopathic, retinal vasculopathy characterized by telangiectasia and yellowish exudation. Both diseases commonly affect males. Here, we report an atypical case of unilateral Eales' disease with co-existing, secondary Coats'-like reaction in a 25-year-old male exhibiting unilateral retinal telangiectasia accompanied by subtle macular edema, extensive peripheral vascular sheathing, and distal non-perfusion in the absence of clinically visible exudation., Methods: Case report., Results: A 25-year-old male presented with painless blurring of vision in the right eye for two months. Best-corrected Snellen visual acuity was 3/60 in the affected eye, with relative afferent pupillary defect observed, and 6/9 contralaterally. Anterior segment examination was unremarkable. Right eye fundoscopy showed aneurysmal telangiectasia at the macula, 360 degrees perivascular sheathing and telangiectasia of peripheral retinal vessels. There was no exudation, neovascularization, tractional membranes, vitritis, retinitis, and choroiditis. Left eye fundus was unremarkable. Optical coherence tomography of the macula revealed disorganization and thinning of the inner retinal layers with minimal intraretinal fluid. Fundus fluorescein angiography showed leakage from the aneurysms but not from the sheathed vessels, and an enlarged foveolar avascular zone. A well-defined zone of non-perfusion and retinal vascular telangiectasia with shunts was observed. Blood investigations ruled out metabolic, infectious, haematological, and autoimmune causes. Carotid Doppler showed no stenosis, and CT angiography of the brain did not show any vascular abnormalities. In view that no cause was identified, a diagnosis of Eales' disease with secondary Coats'-like reaction was made. Treatment consisted of FFA-guided laser photocoagulation to the areas of non-perfusion. Intavitreal anti-vascular endothelial growth factor injection was not done in view of poor visual prognosis. The condition and visual acuity remained stable during 18 months of follow-up., Conclusion: This report highlights an atypical case of Eales' disease with co-existing, secondary Coats'-like reaction., (© 2024. The Author(s).)

Purpose: To evaluate short-term outcomes of intravitreal injection of aflibercept 8 mg for neovascular age-related macular degeneration (nAMD)., Study Design: Retrospective, interventional case series., Methods: We retrospectively studied 35 eyes of 34 consecutive patients with nAMD, assessing best-corrected visual acuity (BCVA), foveal thickness (FT), and central choroidal thickness (CCT) before and 4 weeks after the initial intravitreal dose of aflibercept 8 mg. The rate of achieving a dry macula and the incidence of intraocular inflammation (IOI) at week 4 were also determined., Results: BCVA showed significant improvement, with significant reductions in FT and CCT 4 weeks after the initial injection of aflibercept 8 mg (all P < 0.01), with a dry macula being achieved in 20 eyes (57.1%). However, 3 eyes (8.6%) developed non-infectious IOI associated with retinal vasculitis, an adverse event not reported previously. The IOI in these eyes was relatively mild and treated with a posterior subtenon injection of triamcinolone acetonide with or without betamethasone eye drops, resulting in amelioration of IOI without any visual loss., Conclusions: Intravitreal aflibercept 8 mg appears to be effective for improving visual acuity and ameliorating exudative changes in eyes with nAMD. However, special attention should be given to the potential development of IOI associated with retinal vasculitis., (© 2024. The Author(s).)

Purpose: To describe unilateral vasculitic central retinal vein occlusion in a young adult whose vision problems preceded systemic symptoms of COVID-19 infection., Methods: Observational clinical case report., Results: A 39-year-old immunocompetent man without prior ocular disease presented for vitreoretinal care complaining of decreasing vision in his right eye for 2 weeks. Headaches, pharyngitis, and coughing began 4 days after his visual symptoms. COVID-19 testing was negative before initial vitreoretinal evaluation and positive afterward. Dilated and tortuous major retinal veins in his right eye had prominent perivascular sheathing. A large subhyaloid hemorrhage spanned the macula. Subretinal hemorrhages were present in areas of sheathing, and diffuse nerve fiber layer hemorrhages were arrayed in the distribution of the radial peripapillary capillary plexus. Laboratory tests for inflammatory diseases were negative. The patient was hospitalized for COVID-19 pneumonia a few days after his initial vitreoretinal evaluation. Pars plana vitrectomy was performed for persistent subhyaloid hemorrhage 8 weeks after his hospitalization. The VA in the patient's right eye improved from counting-fingers to 20/30 postoperatively., Conclusion: The patient's findings are consistent with an atypical central retinal vein occlusion which we hypothesize to be of vasculitic origin because of prominent associated retinal phlebitis and venous sheathing. Concomitant subhyaloid, nerve fiber layer, and subretinal hemorrhages involved the superficial and deep retinal vascular complexes. The patient's COVID-19-related hospitalization and systemic management delayed surgical management of his subhyaloid hemorrhages, but a good visual result was achieved despite persistence of macular preretinal blood for 3 months.

Background: Retinal vasculopathy with cerebral leukoencephalopathy and systemic manifestations (RVCL-S) is a rare autosomal dominant systemic microvascular disorder attributed to TREX1 (three-prime repair exonuclease-1) gene mutations, often proned to misdiagnosed., Methods: We reported a case of RVCL-S coexisting with systemic lupus erythematosus due to a mutation in the TREX1 gene. This study provided a summary and discussion of previously documented cases related to TREX1 mutations or RVCL-S., Results: A 39-year-old female patient visited the clinic due to progressive memory loss and speech difficulties. Magnetic resonance imaging results showed corpus callosum atrophy and multiple subcortical calcifications in both brain hemispheres. Genetic testing revealed a TREX1 gene mutation (c.294dupA). Treatment with immunosuppressive therapy for 2 months led to improvements in communication and mobility. We also summarized previously reported cases providing an overview of TREX1 gene mutation or RCVL-S., Conclusion: Our case establishes a compelling foundation for future RVCL-S diagnosis and treatment paradigms. Notably, conducting systemic immunity screening in patients with RVCL-S emerges as a strategic approach to prevent potential diagnostic oversights., (© 2024 The Author(s). Immunity, Inflammation and Disease published by John Wiley & Sons Ltd.)

A 60-year-old female with known seropositive rheumatoid arthritis and other co-morbids, presented with right eye hypertensive retinopathy and exudative macroaneurysms. Over the years, she developed vitreous haemorrhage, macula oedema and full thickness macula hole. Fluorescein angiography showed macroaneurysms and ischaemic retinal vasculitis. Initial diagnosis was thought to be hypertensive retinopathy with macroaneurysms and retinal vasculitis secondary to rheumatoid arthritis. Laboratory investigations did not support other causes for macroaneurysms and vasculitis. Hence, delayed diagnosis of IRVAN syndrome was made after detailed review of clinical findings, investigations, and angiographic evidence. Our understanding of IRVAN continues to evolve in the face of challenging presentations. To our knowledge, this is the first reported case of IRVAN associated with rheumatoid arthritis.

Purpose: To describe a rare case of bilateral frosted branch angiitis with macular exudates in a 5-year-old boy presumed to be secondary to an Epstein-Barr virus (EBV) infection., Observations: A 5-year-old boy presented with acute onset blurring of vision in both eyes, the best corrected visual acuity was 3/60 in both eyes. The anterior segment was unremarkable, fundus showed the presence of perivascular sheathing involving the macular vessels, exudates, and subretinal fluid at the fovea (OU). Serology was positive for EBV. Swept-source optical coherence tomography angiography showed areas of flow void in the superficial and deep capillary plexus that disappeared with reduction of inflammation following treatment with intravenous, oral and topical steroids, and oral valacyclovir., Conclusions: Frosted branch angiitis can present following an EBV infection.

This was a retrospective chart review in Western Australia, Australia.To describe the diagnosis, management, and treatment outcomes of ocular TB in Western Australia (WA).This was a retrospective review of ocular TB cases in WA from 2007 to 2018 with a minimum 2-year follow-up upon completion of anti-TB therapy (ATT).A total of 44 patients were referred to WA TB clinic. Ten were excluded from the analysis of treatment response; 34 met the inclusion criteria, of whom 97.1% were born overseas. No patients had symptomatic extraocular TB. Chest X-ray showed prior pulmonary TB in 11.7% of patients ( n = 4). All patients were treated with three or four ATT drugs. The most common ocular TB manifestation was retinal vasculitis (23.5%). Full resolution of ocular inflammation following ATT occurred in 66.7% ( n = 22), and reduced ocular inflammation requiring only topical steroid treatment was seen in 21.2% ( n = 7). Treatment failure occurred in 12.1% ( n = 4). Side effects were reported in 45.6% of patients, with gastrointestinal symptoms most common (27.2%).Our study is the first Australian study examining the management of ocular TB. Our study highlights the challenges in diagnosing TB ocular disease in a low-endemicity setting and the importance of the collaboration between uveitis and TB subspecialists..

Purpose: To report the clinical features of cytomegalovirus (CMV) retinitis with panretinal occlusive vasculitis., Methods: Retrospective case series., Results: Four eyes in 3 non-HIV patients (male: female = 3:0) were included. Previous medical history included diabetes mellitus (n = 2), age-related macular degeneration (n = 1), and Multiple myeloma under chemotherapy (n = 1). All patients were treated with oral valganciclovir and intravitreal ganciclovir. Slow resolution of retinitis related retinal opacification was noted in all 4 eyes. Two eyes had anti-viral agents discontinued despite the persistent retinitis related opacification and the lesions slowly resolved in the following months. The final decimal visual acuity was equal to or worse than 0.02 in 3 of the 4 eyes., Conclusion: In eyes of CMV retinitis with panretinal occlusive vasculitis, rapid resolution of retinitis lesions is an unreliable sign evaluating the therapeutic efficacy of anti-viral agents. Besides, despite treatment of anti-viral agents, deteriorating vascular occlusion may further endanger macular function.

Purpose: To report a case of bilateral retinal vasculitis successfully managed with adalimumab in a patient with synovitis, acne, pustulosis, hyperostosis, and osteitis (SAPHO) syndrome., Case Report: SAPHO syndrome was diagnosed in a 48-year-old female with bilateral blurred vision refractory to steroid eye drops. A preliminary ophthalmic examination revealed bilateral intermediate uveitis with vitreous opacity, and fluorescein angiography (FA) revealed dye leakage from peripheral retinal vessels. Because oral antirheumatic drugs were ineffective in treating her osteitis, her internist prescribed adalimumab, which resulted in rapid normalization of C-reactive protein and improvement of osteitis. Following treatment with adalimumab for 5 months, FA revealed significant improvement of retinal vasculitis. This is the first report on the use of adalimumab for retinal vasculitis associated with SAPHO syndrome., Conclusion: We described a rare case of retinal vasculitis associated with SAPHO syndrome. Adalimumab treatment was effective in treating both osteitis and retinal vasculitis.

Purpose: Our aim was to report a comprehensive multimodal imaging case of unilateral frosted branch angiitis in a 40-year-old White woman., Methods: Case report involving clinical examination, ultra-wide field fundus photograph, ultra-wide field fluorescein angiography, optical coherence tomography (OCT) and OCT angiography., Results: A 40-year-old patient presented with unilateral acute vision loss. On fundus examination, extensive retinal veins sheathing, macular edema, and vascular congestion were observed while ultra-wide field fluorescein angiography revealed a hyperfluorescent "hot" optic disc and blood retinal barrier disruption. Optical coherence tomography angiography displayed foveal avascular zone enlargement and excluded papillary neovascularization. Extensive laboratory workup for infectious, autoimmune, and inflammatory disorders was negative; thus, a diagnose of acute idiopathic unilateral frosted branch angiitis was made. Intravitreal injection of dexamethasone implant was administered with a good clinical response., Conclusion: Multimodal imaging is crucial to correctly diagnose and treat frosted branch angiitis. To the best of our knowledge, the use of optical coherence tomography angiography as a complementary tool to the diagnostic process in frosted branch angiitis has been described in literature just once as a photographic essay of cytomegalovirus-related frosted branch angiitis 1 and it might be of great value for better characterizing clinical features of this disorder and for following disease activity in a noninvasive fashion.

Purpose: We studied Korean patients with Eales' disease to document clinical features, long-term outcomes, and explore its association with TB, given South Korea's high TB burden., Methods: We retrospectively reviewed medical records of Eales' disease patients for clinical characteristics, long-term outcomes, and its association with TB., Results: Among 106 eyes, the average age of diagnosis was 39.28 years, with 82.7% male and 58.7% having unilateral involvement. Patients who underwent vitrectomy showed greater long-term improvement in visual acuity ( P  = .047), while those with glaucoma filtration surgery showed less improvement ( P  = .008). Having glaucoma through disease progression was associated with poor visual outcomes (odds ratio=15.556, P  < .02). 27 out of 39 patients (69.23%) who underwent IGRA screening tested positive for TB., Conclusions: In Korean patients with Eales' disease, we observed male predominance, unilateral presentation, older age of onset, and a link with TB. Timely diagnosis and management should be considered to maintain good vision in patients with Eales' disease.

Purpose: To report a rare case of cytomegalovirus (CMV)-associated non-necrotizing viral retinopathy, occlusive retinal vasculitis, papillitis, and retinal neovascularization in a young 41-year-old woman., Methods: Case report., Results: The patient presented with features of papillitis, peripapillary cotton-wool spots, pre-retinal hemorrhages, and occlusive vasculitis. Her visual acuity was 20/100 in the left eye. She developed a worsening of the disease upon initiation of systemic corticosteroids. Her serum immunoglobulins (Ig) (both IgG and IgM) were highly positive for CMV. Anterior chamber paracentesis was positive for CMV DNA using real-time polymerase chain reaction. After stopping systemic corticosteroids, she was initiated on oral valganciclovir, with rapid resolution of the vasculitis and cotton-wool spots. After three months, the patient developed retinal neovascularization and underwent pan-retinal photocoagulation. However, her uveitis was inactive, and her visual acuity improved to 20/25., Conclusions: Non-necrotizing viral retinopathy has been associated with either varicella zoster virus (VZV) or herpes simplex virus (HSV). Our case highlights that CMV can also lead to non-necrotizing retinopathy and must be suspected in patients who may be negative for VZV and HSV. Appropriate anti-viral treatment can prevent severe vision loss in these patients.

Inflammatory changes in the retinal vessels can be attributed to a wide range of etiologies. These include infections, intraocular and systemic autoimmune processes, general diseases and iatrogenic factors. As the endothelium of the retinal capillaries forms the inner blood-retinal barrier, a disruption of this structure is directly associated with consequences for the fluid electrolyte balance of the retina. Clinical sequelae can include leakage of the retinal vessels and macular edema, which are often functionally threatening and significantly reduce the quality of life of patients. As the eye can be affected as an "index organ", a work-up of the patient by the ophthalmologist is of great importance. In the age of "precision medicine", efforts are being made to gain new insights into the pathogenetic mechanisms of vasculitis through "omics" in order to develop innovative treatment concepts., (© 2024. The Author(s).)

Objective: To describe a case of bilateral retinal vasculitis due to presumed sarcoidosis and rickettsial retinitis complicated with neovascularization with tear biomarker analysis., Methods: A retrospective case report., Results: A 16-year-old male presented with bilateral retinal vasculitis and retinitis in both eyes with inferotemporal quadrant neovascularization in the right eye. Multimodal imaging revealed the presence of active inflammation in both eyes. Weil Felix test was positive with raised ACE levels. This patient was treated with local and systemic steroids, doxycycline, and laser photocoagulation followed by oral methotrexate therapy which resulted in clinical resolution with recovery of visual acuity. Tear biomarker analysis showed raised sICAM-1 and MMP-9 levels in both eyes which significantly reduced following treatment., Conclusion: Ocular sarcoidosis with rickettsial infection is a rare association. Tear biomarkers correlated well with clinical and imaging manifestations. High index of suspicion and aggressive anti-inflammatory therapy can help control inflammation and restore good vision., Competing Interests: Declaration of conflicting interestsThe authors declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

A 13-year-old Black male patient with a history of Kikuchi-Fujimoto disease (KFD) and sickle cell trait presented with acute painless vision loss and no light perception vision (NLP) in his left eye. The examination was indicative of occlusive retinal vasculitis with near total central retinal artery occlusion (CRAO). He was started on oral steroids with dramatic reperfusion and improvement of the retinal hemorrhages. However, his vision remained at NLP. Oral steroids were tapered, and rituximab infusion was initiated. While ocular involvement is uncommon in KFD, vision-limiting complications, such as occlusive retinal vasculitis, ophthalmic artery occlusion, and CRAO can occur. Early systemic immunosuppression is key in achieving rapid remission. [ Ophthalmic Surg Lasers Imaging Retina 2024;55:235-239.] .

Background: Retinal vasculitis is an inflammatory condition that affects the retinal blood vessels., Summary: It can manifest as an idiopathic disorder or be secondary to various infectious or non-infectious diseases, mimicking syndromes, isolated ocular disorders, or drug-induced reactions. Recognizing its distinctive features is crucial for early diagnosis and accurate treatment. This review aimed to demonstrate the variety of tools available to detect disease activity, assess complications, measure the extent of retinal damage, and guide therapy effectively., Key Message: This review article highlights the use of multimodal imaging in the comprehensive evaluation of retinal vasculitis., (© 2024 S. Karger AG, Basel.)

Introduction: Idiopathic retinal vasculitis, aneurysms, and neuroretinitis (IRVAN) syndrome is a rare clinical entity affecting young healthy individuals. Treatment primarily involves pan retinal photocoagulation (PRP) to capillary non perfusion areas. Intravitreal anti-VEGF or steroids are given in the presence of macula edema. Oral steroids do not alter the course of the disease. Arterial occlusions have been reported in IRVAN., Methods: Retrospective case review., Result: A twenty seven year old male presented to us with mild blurring of vision for one week. His BCVA was OU 20/20. Anterior segment examination was normal. Fundus examination showed bilateral disc aneurysm with OS arterial aneurysm along the inferior arcade. Fundhus fluorescein angiography and OCT angiography were confirmatory of the disc and retinal aneurysm. Capillary non perfusion (CNP) areas were noted in the periphery. Two days later he presented with paracentral scotoma in his left eye which was confirmed by Amsler chart. Fundus, OCT and OCTA were confirmatory of Paracentral Acute Middle Maculopathy (PAMM). The retinal aneurysm had increased in size from 333 micron diameter to 566 micron diameter. Panretinal photocoagulation to the CNP areas was done and intravitreal antiVEGF was given. At 6 months follow up, retinal aneurysm had disappeared., Discussion: Our case describes a unique event with sudden increase in size of the aneurysm leading to acute blockage in the deep capillary plexus thus being the first report of PAMM in IRVAN. The patient was treated with PRP and intravitreal anti-VEGF for the enlarging aneurysm which reduced in size within a week., Competing Interests: Declaration of conflicting interestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Aim: To describe and correlate electroretinographic responses with clinical and angiographic findings in retinal vasculitis (RV)., Methods: Medical records of patients with diagnosis of RV at a tertiary eye centre from December 2017 to May 2021 were reviewed. Cases in which fluorescein angiography (FFA) and full field electroretinography (ffERG) were done within 1 month were included. FFAs were graded according to the Angiography Scoring for Uveitis Working Group from 0 to 40, where 0 is normal. A novel ffERG grading system was implemented where individual waves were graded for timing and amplitude and general ffERG score was determined with 6 being a perfect score., Results: 20 patients (34 eyes) were included. Mean age was 43.9±19.8 years; 70% were female. Median best-corrected visual acuity was 0.8 (0.08-1). Mean FFA score was 12.6±6.5. Median general ffERG score was 5 (0-6). 68% and 91% of eyes had responses with general ffERG scores ≥5 and 4, respectively. Flicker timing was most commonly affected.FFA scores weakly correlated with delayed photopic cone b-wave and flicker timing (p=0.03 and 0.016, respectively). Vitreous haze moderately correlated with delayed cone b-wave timing (p<0.001), delayed flicker timing (p=0.002) and weakly correlated with lower flicker amplitude (p=0.03). Underlying systemic disease was associated with poor ffERG responses., Conclusion: In this study, RV was not frequently associated with severe global retinal dysfunction Higher FFA scores, and vitreous haze grading were weakly, but significantly, correlated with cone-generated ffERG responses., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2023. No commercial re-use. See rights and permissions. Published by BMJ.)

Background: Intraocular inflammation is common after anterior or posterior segment surgery. They typically manifest either as non-infectious inflammation of the anterior or posterior segment, known as toxic anterior or posterior segment syndrome (TPSS), or as sterile or infective endophthalmitis. In this report, we describe a rare case of TPSS following vitreoretinal surgery, presenting as hemorrhagic retinal vasculitis., Case Presentation: A 58-year-old male diagnosed with a left eye acute rhegmatogenous retinal detachment underwent an uneventful primary pars plana vitrectomy with silicone oil endotamponade on the same day of presentation. At presentation, there were no signs of intraocular inflammation, and his visual acuity in the affected eye was 20/200., Results: The retina was well-attached with silicone oil in place on the first post-operative day. Along the inferior retinal periphery, a hemorrhagic occlusive vasculitis was observed. Clinical examination revealed retained intraocular cotton fiber along the inferotemporal quadrant over the retinal surface. In addition to the standard post-operative medications, a course of systemic steroids (40 mg per day of Prednisolone tablets) was started. At the end of the first post-operative week, clinical signs of hemorrhagic retinal vasculitis were beginning to resolve, and by the end of the fourth post-operative week, they had completely resolved., Conclusion: This report describes an unusual diagnosis of TPSS after vitreoretinal surgery, most likely due to the presence of an intraocular cotton fiber. This excessive inflammation of the posterior segment usually responds to a course of topical and systemic steroids., (© 2023. The Author(s).)

Purpose: To investigate the frequency, clinical findings and outcomes of occlusive retinal vasculitis in patients with multiple sclerosis (MS)., Methods: A retrospective case series., Results: During the period between January 2000 and December 2021, we identified 24 patients who were diagnosed to have uveitis associated with MS. Among them, four (16.6%) patients presented with bilateral occlusive retinal vasculitis who were diagnosed to have MS prior to presentation. All patients were treated successfully with a combination of systemic corticosteroids combined with mycophenolate mofetil. In addition, scatter laser photocoagulation was applied to the ischemic retina in all eyes., Conclusions: Early recognition and prompt treatment with systemic immunosuppressive agents and scatter laser photocoagulation prevent complications and improve outcomes in MS patients with occlusive retinal vasculitis.

Purpose: Wide-field fluorescein angiography is commonly used to assess retinal vasculitis (RV), which manifests as vascular leakage and occlusion. Currently, there is no standard grading scheme for RV severity. The authors propose a novel RV grading scheme and assess its reliability and reproducibility., Methods: A grading scheme was developed to assess both leakage and occlusion in RV. Wide-field fluorescein angiography images from 50 patients with RV were graded by four graders, and one grader graded them twice. An intraclass correlation coefficient (ICC) was used to determine intraobserver-interobserver reliability. Generalized linear models were calculated to associate the scoring with visual acuity., Results: Repeated grading by the same grader showed good intraobserver reliability for both leakage (ICC = 0.85, 95% CI 0.78-0.89) and occlusion (ICC = 0.82, 95% CI 0.75-0.88) scores. Interobserver reliability among four independent graders showed good agreement for both leakage (ICC = 0.66, 95% CI 0.49-0.77) and occlusion (ICC = 0.75, 95% CI 0.68-0.81) scores. An increasing leakage score was significantly associated with worse concurrent visual acuity (generalized linear models, β = 0.090, P < 0.01) and at 1-year follow-up (generalized linear models, β = 0.063, P < 0.01)., Conclusion: The proposed grading scheme for RV has good to excellent intraobserver and interobserver reliability across a range of graders. The leakage score related to present and future visual acuity., (Copyright © 2023 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of the Opthalmic Communications Society, Inc.)

Background: With the popularity of coronavirus disease 2019 (COVID-19) vaccination, some rare ocular adverse events have gradually been reported. This report shows a rare case of retinal vasculitis following COVID-19 vaccination., Description: A 37-year-old male complained of unilateral severe vision loss 13 days after the first dose of an inactivated COVID-19 vaccine. The initial eye examination showed gray-white edema in the macula, and scattered patchy gray-white lesions in the peripheral retina, accompanied by peripheral retinal small vessel occlusion. After glucocorticoid treatment, the gray-white lesions gradually subsided. However, due to the initial severe damage in the fundus, macular and peripheral retinal lesions were significantly atrophied, and the vision recovery was poor., Conclusion: The ocular adverse event in this report may be related to the inactivated COVID-19 vaccination, however, it is difficult to rule out the accidental rare adverse events after a large number of vaccinations.

Purpose: To report two cases of bilateral retinal vasculitis in adolescents following COVID-19 vaccination., Study Design: Case report., Results:  We report the first two cases of retinal vasculitis in adolescents following COVID-19 vaccinations. Both patients received recent second-dose COVID-19 vaccinations (7 weeks and 4 weeks respectively), and presented with bilateral retinal vasculitis and vitritis. Investigations did not reveal other causes of retinal vasculitis. Both patients' retinal vasculitis settled with a short course of oral prednisolone., Conclusion: Although rare, the temporal association between vaccination, bilateral eye involvement, and the absence of alternative infective or inflammatory causes, makes this a plausible etiology. mRNA vaccinations may cause an autoimmune reaction via host antigenic mimicry, and systemic vasculitis has previously been described. We believe that a short interval between COVID-19 vaccination doses might be a risk factor for the development of retinal vasculitis in adolescents, and clinicians should be aware to elicit vaccination history.

A 23-year-old man presented with the blurring of vision in the left eye for 4 days. Best-corrected visual acuity was 6/6 N6 in both eyes. Examination revealed an unremarkable right eye while the left eye showed occlusive retinal vasculitis with no retinitis, choroiditis, or macular involvement. Fundus fluorescein angiography confirmed the same. History revealed the patient had received 2nd dose of Covishield vaccination 4 weeks before the onset of symptoms. Blood investigations were negative for infectious or any systemic autoimmune disease. Serum homocysteine and serum CMV IgG levels were grossly increased while tests for antiphospholipid syndrome were weakly positive. He responded well to a combination of intravitreal and oral antivirals, oral steroids for vasculitis and tablets Clopilet and Homin. This case is extremely intriguing in terms of the involvement of the adenoviral vector vaccine either as a causative factor or being just a coincidental finding.

Purpose: To evaluate the efficacy of infliximab (IFX, 5-10 mg/kg) (Group 1) and tocilizumab (TCZ, 4-8 mg/kg) (Group 2) infusions in non-infectious retinal vasculitis (RV) using Angiographic Scoring for the Uveitis Working Group fluorescein angiography (FA) scoring system., Methods: Records of 14 patients (24 eyes) in Group 1 and 8 patients (11 eyes) in Group 2 were retrospectively evaluated to assess visual acuity (VA), anterior chamber cell and flare, vitreous haze, central subfield thickness (CST), and FA scoring at baseline and 6 months of follow-up. The measurements were employed to grade in each group., Results: In Group 1 and 2, respectively, there was no underlying disease in 9 (60%) and 3 (42.9%) patients. Three (42.9%) patients in Group 2 had juvenile idiopathic arthritis (JIA) as the most common identified cause. Mean improvement in VA (log MAR) and CST were 0.04 ± 0.14 and 40.3 ± 78.5 µm in Group 1; 0.04 ± 0.09 and 47.3 ± 82.3 µm in Group 2, respectively. Mean FA scores were significantly reduced from 12.4 ± 5.2 and 11.6 ± 4.4 at baseline to 6.4 ± 5.0 and 5.8 ± 3.9 at 6-month in Group 1 and 2, respectively. In Group 2, 9 eyes of 6 patients (75%) had the history of IFX use prior to TCZ initiation. There was no significant safety concern requiring treatment discontinuation during the follow-up in either group., Conclusion: IFX and TCZ infusions showed statistically significant improvement of non-infectious RV as shown by ASUWOG FA Scoring System. TCZ, as well as IFX, appeared to be effective treatment options for non-infectious RV., (© 2022. The Author(s), under exclusive licence to The Royal College of Ophthalmologists.)

Background: Eales' disease is an idiopathic peripheral retinal vasculopathy characterized by retinal phlebitis, ischemia, retinal neovascularization, and recurrent vitreous hemorrhages. But CRAO is an unusual presentation., Case Presentation: A 27-year-old healthy female nurse of Indian descent presented with sudden vision loss in her right eye upon awakening. Central retinal artery occlusion (CRAO), combined with mild central retinal vein occlusion (CRVO), was diagnosed. During the second of three consecutive sessions of hyperbaric oxygen treatments, her vision rapidly improved. One week later, she developed peripheral phlebitis in the same eye. Infectious, inflammatory, and hematologic etiologies were excluded. The systemic evaluation was normal except for a positive Mantoux tuberculin skin test. Following systemic steroidal treatment, she experienced gradual improvement of her vasculitis. Two weeks later, mild retinal phlebitis appeared in her left eye. Eales' disease was diagnosed after the exclusion of other diseases., Conclusion: This is an unusual Eales' disease case, which presented as combined CRAO with mild CRVO. The association of CRAO and Eales' disease is reported here for the first time, to our best knowledge., (© 2023. The Author(s).)

Purpose: To present a case of frosted branch angiitis associated with an exacerbation of mixed connective tissue disease (MCTD)., Methods: Single case report., Results: A 31-year-old woman presented with a flare of her long-standing MCTD after a change in her immunosuppressive medications. She developed blurred vision and floaters first in the left eye but eventually in both eyes. Fundoscopy showed patchy perivascular sheathing of tertiary branch venules surrounded by retinal hemorrhages characterized as frosted branch angiitis. The patient's MCTD symptoms and retinal vasculitis improved with continued immunosuppressive therapy. At 1-month follow-up, her visual acuity had improved to 20/20 bilaterally with complete resolution on fundoscopy., Conclusion: To the authors' knowledge, this is the first report of frosted branch angiitis seen in association with MCTD. The improvement in the patient's visual acuity and fundoscopic findings in this case supports the role of immunosuppressive therapy to treat secondary frosted branch angiitis associated with an autoimmune condition such as MCTD. However, it is recommended that a comprehensive medical workup is performed to exclude an infective cause, particularly in immunocompromised patients.

Purpose: To discuss diagnosis and management in the case of a patient presenting with bilateral ischemic retinal vasculopathy associated with a previously undiagnosed IgA nephropathy., Case Report: In 2021, a 35-year-old male presented with a sudden onset asymmetric bilateral (OU) visual loss. Best-corrected visual acuity (BCVA) was 20/200 OD, and no light perception (NLP) OS with an associated relative afferent pupillary defect (RAPD). Slit-lamp examination (SLE) confirmed normal anterior segment anatomy OU. Indirect ophthalmoscopy and subsequent fluorescein angiography (FA) confirmed the presence of bilateral arterial attenuation, telangiectatic lesions, associated perivascular sheathing, and capillary leakage. Spectral domain optical coherence tomography (OCT) showed multiple areas of thinning of the inner retina. This constellation of diagnostic findings was highly suggestive of a bilateral ischemic retinal vasculopathy with an inflammatory vasculitis. Based on a high index of suspicion for a systemic etiology, nephrology was consulted, and a diagnosis of IgA nephropathy was confirmed by renal biopsy. Systemic immunomodulatory therapy was initiated., Conclusion: Although it is among the most commonly occurring forms of glomerulonephritis leading to renal failure, IgA nephropathy rarely presents with a bilateral retinal vasculopathy. Schölein - Henoch purpura, the other primary disease associated with glomerular IgA deposition, may be indistinguishable from primary IgA nephropathy. A comprehensive retinal examination with multimodal functional and structural ophthalmic diagnostic testing in conjunction with renal biopsy was needed to confirm the diagnosis. It is critical to include primary renal pathology when formulating a differential diagnosis for cases of bilateral retinal vasculitis, particularly in young otherwise healthy patients.

Purpose: To report a severe bilateral frosted branch angiitis associated with acute onset of systemic dermatomyositis., Method: History and clinical examination, laboratory evaluation, fundus' and skin's color pictures, fluorescein angiography (FA), optical coherence tomography (OCT)., Results: A 32-year-old female was referred for a bilateral frosted branch angiitis and vitreitis, with skin rash, muscle pain, weakness, severe headache, compatible with dermatomyositis. After an initial improvement following an aggressive treatment by systemic steroids and mycophenolate mofetil, she was switched to intravenous infliximab (RemicadeTM, Janssen Biotech, Inc. USA) due to lack of efficacy. Nine months later, her visual acuity was improved up to 20/20 in OU, with both retinal vasculitis and vitreitis being solved., Conclusion: Dermatomyositis is a severe multiorgan disease which might severely involve eye structures. Anti-TNF-Alpha agents, particularly Infliximab, might offer a rapid control and long-term remission.

An 8-year-old girl presented with a subretinal abscess after strabismus surgery. This was treated successfully with medial rectus suture removal, pars plana vitrectomy, intravitreal antibiotics, and intravenous antibiotics. Recovery was complicated by acute post-infectious retinal vasculitis after tapering high-dose corticosteroids, requiring an extended corticosteroid regimen over 2 months until resolution. [ J Pediatr Ophthalmol Strabismus . 2023;60(3):e26-e30.] .

Purpose: To report a case of IRVAN in a 13-year-old girl responding well to Adalimumab and Azathioprine., Results: A 13-year-old girl presented to us with central scotoma for a duration of 10 months. She was treated earlier with oral steroids with poor response. Fundus examination revealed features of IRVAN. She was treated with intravitreal dexamethasone implant in both eyes with oral Mycophenolate Mofetil (MMF) with transient response to it. So she was switched over to subcutaneous Adalimumab 40 mg once in 2 weeks and oral Azathioprine 50 mg BD. The disease activity was well controlled with the current regime., Conclusion: Though various treatment modalities have been described in literature for the treatment of IRVAN. This is the first case of IRVAN to be treated with Adalimumab along with Azathioprine to be reported.

Aim: To evaluate the effect of repository corticotropin injection (RCI) on regulatory T cell population in patients with noninfectious retinal vasculitis., Patients and Methods: Patients with active noninfectious retinal vasculitis were included in a prospective nonrandomized open-label study., Results: Eighteen patients (33 eyes) were included in the study. Eleven (61.1%) patients [20 (60.6%) eyes] and 7 (38.9%) patients [13 (33.3%) eyes] were in the responsive and non-responsive groups, respectively. We did not find any statistically significant difference within the PPP-R group, within the PPP-NR group, or between these two groups in regard to regulatory T cell population. No significant systemic or ocular complications were found., Conclusion: RCI may be a complementary treatment in patients with non-infectious retinal vasculitis with or without uveitis. This study did not demonstrate an increase in regulatory T cell population in patients with noninfectious retinal vasculitis.