IDIOPATHIC RETINAL VASCULITIS, ANEURYSMS, AND NEURORETINITIS (IRVAN) SYNDROME: CASE REPORTS
Idiopathic retinal vasculitis, aneurysms, and neuroretinitis (IRVAN) syndrome is a very rare clinical entity, dissociated from systemic conditions and commonly seen in young female individuals. Its etiology is unknown and the disease is characterized by peculiar vascular features, often bilateral and best visualized on fluorescein angiography (AF). Treatment is aggressive and based on corticosteroids, immunosuppressors, laser and anti VEGF injections.
Three patient were identified with inflammatory and vascular features compatible with IRVAN. All of them were negative for infectious, rheumatological and thrombophilia diseases and responded to the treatment implemented. Age ranged from 13 to 33 years, by the time of diagnoses, being one of them a male individual. All cases happened bilaterally. Most common ocular manifestations observed were: mild vitreous cells, disc edema, retinal exudation, vasculitis and arterioles aneurysms. AF showed retinal ischemia in the three cases. Because of those findings, patients were aggressively treated with corticosteroid therapy, immunosuppression panretinal photocoagulation and anti-VEGF intravitreal injections were performed if necessary.
Considering the high risk of neovascularization over time, the disease prognosis depends on your early recognition and offensive approach is crucial for a better visual outcome.
irvan syndrome
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Ana Carolina Andrade de Araújo Lima, Renata Nobre Maia, Danuza Oliveira Machado Azevedo, Daniel Vitor Vasconcelos-Santos