Occlusive Retinal Vasculitis in a Pediatric Patient With Kikuchi-Fujimoto Disease and Sickle Cell Trait.

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.].

Characteristics, Etiology, and Clinical Outcome of Retinal Vasculitis in Tertiary Hospital in Thailand.

OBJECTIVE: To analyze characteristics, etiology, and outcome of retinal vasculitis in Central Thailand. METHODS: A retrospective cohort study. RESULTS: Retinal vasculitis was found in 10% of uveitis, 74 from 741 uveitis, noninfectious (64.9%) and infectious group (35.1%). The most common cause was Behcet's disease (48.6%). Behcet's disease was the most common cause of all types of vascular leakage on angiography, including capillary (80.4%), venous (56.3%), and arterial leakage (56%). Final visual acuity was 0.86 ± 0.97 logMAR. Cataract was the most frequent complication (42.5%). Acute clinical course (p = .025) and retinal neovascularization (p = .031) were associated with infectious group. Forty-three percent of vasculitis complicated by ischemia required photocoagulation (33%) and anti-VEGF injection (17%). Furthermore, 17% of vasculitis underwent vitrectomy. CONCLUSION: One-half of the retinal vasculitis in Central Thailand were Behcet's disease. Acute onset and retinal neovascularization may suggest infectious etiology. Retinal ischemia should be cautious and undergo early interventions to prevent sight-threatening complications.

Toxic posterior segment syndrome with retinal vasculitis likely caused by intraocular cotton fiber after vitreoretinal surgery - a case report.

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.

Bilateral Retinal Vasculitis as Initial Presentation of Systemic Lupus Erythematosus with Secondary Antiphospholipid Syndrome.

BACKGROUND Systemic lupus erythematosus (SLE) is a multisystem autoimmune disease of undefined etiology with a relapsing and remitting course. Lupus retinopathy is reported in around 10% of patients with SLE; however, it is rarely the initial presenting feature of the disease. We report a unique case of bilateral retinal vasculitis as the initial presentation of SLE with secondary antiphospholipid syndrome (APS). CASE REPORT A 34-year-old man, previously healthy, presented to the eye clinic for the first time with painless reduced vision for 3 weeks. A review of systems revealed generalized fatigue, myalgia, arthralgias, and weight loss of around 10 kg in the last 3 months. On ophthalmic examination, his visual acuity was reduced bilaterally, more in the right eye. A fundus exam revealed bilateral diffuse multiple cotton-wool spots, dot and blot hemorrhage covering the posterior pole, and venous congestion and beading. In addition, there was cystoid macular edema (CME) in the fovea of both eyes, and fundus fluorescein angiography (FFA) showed bilateral areas of peripheral and macular hypo-fluorescence, multiple hyper-fluorescent knob-like aneurysmal dilatations, and vascular leaking and staining. He was diagnosed with SLE by the rheumatology team based on the clinical presentations and laboratory investigations. The patient was managed with intravenous methylprednisolone and discharged on oral prednisone with a tapering regimen. Eighteen months after, he reported significant improvement in his vision with regular follow-ups. CONCLUSIONS Ocular manifestations can be the initial presentation of SLE and can lead to serious ocular complications. Early diagnosis and proper management are essential and require cooperation between rheumatologists and ophthalmologists.

Central retinal artery occlusion as a presenting symptom in Eales' disease: a case report.

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.

FROSTED BRANCH ANGIITIS ASSOCIATED WITH MIXED CONNECTIVE TISSUE DISEASE.

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.

Subretinal Abscess Complicated by Post-infectious Retinal Vasculitis Following Strabismus Surgery.

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.].

Retinal Vasculitis Following COVID-19 Vaccination: Causation or Coincidence?

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.

Rituximab, Intravitreal Bevacizumab and Laser Photocoagulation for Treatment of Macrophage Activation Syndrome and Retinal Vasculitis in Lupus: A Case Report.

Systemic lupus erythematosus (SLE) most commonly manifests as mild to moderate disease with severe manifestations such as diffuse alveolar hemorrhage, central nervous system vasculitis, macrophage activation syndrome (MAS) or retinal vasculitis (RV) with visual disturbances occurring in a significantly smaller proportion of patients, most of whom have a poor outcome. Macrophage activation syndrome and RV are insufficiently early and rarely recognized presentations of lupus-consequently there are still no treatment recommendations. Here we present the course of diagnosis and treatment of a patient with an SLE flare that resulted in both life-threatening disease (MAS) and vision-threatening disease (RV). The patient was successfully treated with systemic immunosuppressives, a high dose of glucocorticoids and rituximab (RTX), in parallel with intraocular therapy, intravitreal bevacizumab (BEV) and laser photocoagulation.

Cocaine Abuse as an Immunological Trigger in a Case Diagnosed with Eales Disease.

Background: Eales disease is a clinical syndrome affecting the mid-peripheral retina with an idiopathic occlusive vasculitis and possible subsequent retinal neovascularization. The disease can develop into visually threatening complications. Case Presentation: We report the case of a 40-year-old Caucasian male with a history of cocaine abuse who presented with blurred vision in the left eye (LE). Fundus examination showed vitreous hemorrhages, peripheral sheathing of venous blood vessels, areas of retinal neovascularization in the LE, and peripheral occlusive phlebitis in the right eye. The full serologic panel was negative except for the heterozygous mutation of factor V Leiden. Clinical and biochemical parameters suggested a diagnosis of Eales disease. Therapy with dexamethasone, 1 mg per kg per day, tapered down slowly over 4 months, and peripheral laser photocoagulation allowed a regression of clinical signs and symptoms. Conclusion: This case shows an uncommon presentation of Eales disease associated with cocaine abuse. Both cocaine abuse and a thrombophilic pattern, as cofactors, might have sensitized the retinal microcirculation on the pathogenetic route to this retinal pathology. Furthermore, in view of this hypothesis, a thorough ocular and general medical history investigating drug abuse and coagulation disorders is recommended for ophthalmologists in such cases.

Case Report: Retinal Vasculitis in Two Adolescents After COVID-19 Vaccination.

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 Curious Case of Occlusive Retinal Vasculitis in a Young Individual Associated with COVID-19 Vaccination.

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.

Occlusive Retinal Vasculitis in Patients with Multiple Sclerosis.

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.

Retinal arterial occlusive vasculitis following IgA nephropathy: A case report.

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.

MULTIMODAL IMAGING OF ACUTE MULTIFOCAL HEMORRHAGIC RETINAL VASCULITIS: A CASE REPORT.

PURPOSE: To report a case of acute multifocal hemorrhagic retinal vasculitis and demonstrate the multimodal imaging. METHODS: Interventional case report. RESULTS: A 54-year-old woman without significant past medical history complained of redness and blurred vision in both eyes. Her visual acuity was counting fingers and 20/60. Mild conjunctival injection, anterior chamber cells, and vitreous haze were noted. Fundus showed multifocal intraretinal hemorrhages. Fluorescein angiography revealed vasculitic process with intraretinal hemorrhage blocking defects and retinal ischemic changes in both eyes. Anterior chamber tap fluid polymerase chain reaction for varicella zoster virus, herpes simplex virus I/II, cytomegalovirus, and Epstein-Barr virus was unremarkable. Rheumatology was consulted and systemic vasculitis was ruled out. Her vision improved to 20/50 and 20/20 after pulse methylprednisolone therapy, oral methotrexate, and prednisolone treatment. CONCLUSION: Acute multifocal hemorrhagic retinal vasculitis can occur in an immunocompetent patient. Multimodal Imaging is useful in the diagnosis and follow-up. Patients could benefit from early and aggressive immunosuppressive therapy.

A Comparative Study between Occlusive and Non-occlusive Retinal Vasculitis: Data from a Referral Center in Tunisia, North Africa.

PURPOSE: To compare clinical profile and visual outcomes of occlusive versus non-occlusive retinal vasculitis (RV). METHODS: A retrospective comparative study. RESULTS: 284 patients were enrolled, including 124 patients with occlusive RV (ORV) and 160 patients with non-occlusive RV (NORV). Patients with ORV were older (p ≤ 10-3), predominantly male (p ≤10-3), with less bilateral involvement (31.5% vs 53,4%; p ≤ 10-3). Infectious RV was more frequently diagnosed in the ORV group than in the NORV group (48.8% vs 32.9%, p = .006). Behçet disease and ocular tuberculosis were the leading causes of ORV. Idiopathic RV, Behçet disease, and sarcoidosis were the most common causes of NORV. Independent predictive factors of poor visual outcome were worse baseline visual acuity in both groups (p = .006 and p ≤ 10-3, respectively), and retinal hemorrhages (p = .048) and optic atrophy (p = .040) in the ORV group. CONCLUSION: Occlusive and non-occlusive RV have distinctive clinical and etiological profile.

Paracentral acute middle maculopathy with occlusive retinal vasculitis in presumed intraocular tuberculosis and hyperhomocysteinemia.

A male patient presented with a sudden visual decline in the right eye (OD). Fundus revealed bilateral vasculitis; OD also showed an occluded inferior retinal vein and a wedge-shaped retinal opacification of the inferior macula and nasal retina. Fluorescein angiography revealed occlusive retinal vasculitis, while optical coherence tomography showed paracentral acute middle maculopathy (PAMM) in the OD. A thorough systemic evaluation revealed hyperhomocysteinemia and a positive Mantoux test. A diagnosis of PAMM with occlusive retinal vasculitis in presumed intraocular tuberculosis and hyperhomocysteinemia was made. Retinal vasculitis improved with oral corticosteroid, intravitreal anti-vascular endothelial growth factor and laser photocoagulation. However, the patient declined antitubercular therapy despite recommendations. This unique report indicates that PAMM may complicate tubercular retinal vasculitis, especially in the presence of systemic hypercoagulable states.