Idiopathic retinal vasculitis, aneurysms and neuroretinitis (IRVAN) is a rare ocular condition with an unresolved aetiology, different treatment practices and a risk of severe vision loss in refractory cases. There is sparse literature on the use of biologics, specifically TNF- α inhibitors, in the management of IRVAN, emphasising the need for additional evidence in this therapeutic area. We present a 49-year-old otherwise healthy Norwegian woman who experienced gradual bilateral visual deterioration and was diagnosed with IRVAN syndrome following extensive systemic and ophthalmological evaluation. Despite treatment with monthly bilateral intravitreal anti-vascular endothelial growth factor (anti-VEGF) injections, which were initially combined with systemic corticosteroids and later with peripheral scatter laser photocoagulation, disease control was not achieved. At nadir, the best-corrected visual acuity (BCVA) was 0.4 in OD and 0.3 in OS. Eight months after treatment initiation, the TNF-α inhibitor adalimumab was introduced in combination with methotrexate, and the subsequent clinical course was characterised by substantial and sustained improvement. The macroaneurysms regressed and the vasculitis subsided, leading to complete resolution of the peripapillary oedema and improvement of visual acuity to 0.9–1.0 bilaterally. At two years, the patient remained stable on adalimumab monotherapy without further intervention. Adalimumab may represent a safe and effective option for managing refractory IRVAN syndrome and could address the underlying pathology. Our findings underscore the importance of individualised therapeutic approaches and advocate further investigation of TNF-α inhibitors as a viable treatment option for IRVAN.

Idiopathic retinitis, vasculitis, aneurysms, and neuroretinitis (IRVAN) syndrome is typically diagnosed after characteristic intravenous fluorescein angiography (IVFA) findings develop. This case demonstrates that identifying a green intravascular and perivascular signal on multicolor fundus imaging may be a potential noninvasive imaging sign for ischemic retinal vasculitis in IRVAN. A 55-year-old female presented with bilateral optic disc edema and subtle peripapillary retinal vascular changes. Multicolor imaging showed a distinct green intravascular and perivascular signal before undergoing IVFA evaluation. Subsequent IVFA ultimately showed peripheral capillary nonperfusion, microaneurysms, and leakage consistent with IRVAN. This case demonstrates the potential utility of multicolor imaging for detecting subtle vascular abnormalities that are not apparent on clinical examination or on traditional color fundus photography. Recognition of this green vessel signal may serve as an imaging sign of vascular wall changes, prompting consideration of IRVAN and guiding timely further angiographic evaluation in patients presenting with atypical optic disc edema or suspected retinal vasculitis.

To report a case of idiopathic retinal vasculitis, aneurysm, and neuroretinitis (IRVAN) syndrome with good response to treatment with Adalimumab in addition to panretinal photocoagulation (PRP). Case report. We describe a case of IRVAN in an adolescent who had been initially treated with PRP and intravitreal injections of Ranibizumab in both eyes. Due to persistent retinal ischemia and vasculitis, treatment with subcutaneous injections of Adalimumab was initiated. After 1.5 years of follow-up, there was resolution of retinal vasculitis, and reversal of some retinal capillary non-perfusion with substantial regression of retinal ischemia. Treatment with Adalimumab may be useful in controlling disease activity in patients with IRVAN syndrome.

Idiopathic retinal vasculitis, aneurysms, and neuroretinitis (IRVAN) syndrome is a rare inflammatory vision-threatening retinal disease of unknown origin. If untreated, it can lead to severe vision loss from exudative maculopathy and neovascular sequelae of retinal ischaemia. Detailed IRVAN case reporting may help to provide new insights. Successful treatment of a pediatric patient with infliximab. A 12-year-old girl with IRVAN syndrome received immunosuppressive and laser photocoagulation therapy without improvements. A shift to treatment with infliximab not only reversed disease symptoms on short term, but also led to a complete and long-term cure of the disease. The patient was followed-up for a period of 3 years and infliximab resulted in complete resolution of retinal and optic nerve head aneurysmal dilations, retinal vasculitis and optic disc swelling, and complete resorption of retinal exudation. Infliximab is safe and effective and should be considered as a primary first-line therapy for IRVAN syndrome.

Idiopathic Retinal Vasculitis, Aneurysms, and Neuroretinitis (IRVAN) syndrome is a rare retinal vasculopathy predominantly affecting young individuals. Characterized by retinal vasculitis, aneurysmal dilations, exudation, and neuroretinitis, IRVAN was initially considered a benign condition. However, emerging evidence highlights its potential for severe vision-threatening complications, emphasizing the need for early diagnosis and intervention. The underlying pathogenesis remains unclear, though inflammatory, vascular, and neuroretinal mechanisms are implicated. While most insights stem from case reports and small cohort studies, IRVAN's clinical presentation varies widely. Affected individuals often experience sudden vision loss, typically due to macular involvement, retinal ischemia, or neovascular complications. Imaging techniques, including fluorescein angiography and optical coherence tomography, are essential for diagnosis, allowing detailed visualization of aneurysmal changes and ischemic progression. Recent discussions have proposed a reclassification of neuroretinitis within the disease framework, suggesting alternative nomenclature emphasizing its vascular pathology. Management strategies focus on reducing ischemia and inflammation. Panretinal laser photocoagulation is the cornerstone of therapy, aiming to mitigate neovascular complications. Corticosteroids, immunosuppressive agents, and biologic therapies play roles in controlling inflammatory components. Anti-Vascular Endothelial Growth Factor (Anti-VEGF) treatments have shown promise, particularly in advanced disease stages. Despite these therapeutic options, IRVAN's unpredictable course necessitates individualized treatment approaches. Although primarily an ocular condition, isolated reports suggest possible systemic associations, including intracranial hypertension and autoimmune markers. Future research should aim to establish standardized diagnostic criteria and therapeutic protocols, enhancing outcomes for affected individuals. This review synthesizes current literature to provide insights into clinical presentation, imaging features, differential diagnoses, and evolving management strategies for this rare disorder.