Adalimumab in the management of refractory idiopathic retinal vasculitis, aneurysms, and neuroretinitis (IRVAN) syndrome: a case report and literature review

Abstract Background 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. Case presentation 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. Conclusions 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.