A Case of Idiopathic Retinal Vasculitis, Aneurysms and Neuroretinitis (IRVAN) Syndrome with Elevated Antinuclear Antibody

Background: A case of idiopathic retinal vasculitis, aneurysms and neuroretinitis (IRVAN) syndrome with elevated antinuclear antibody level is described. Case Report: A 10-year-old girl noted decreased vision in both eyes. Her corrected visual acuity was 0.7 in the right eye and 1.5 in the left. Vitreous hemorrhage was noted in both eyes. In the left eye, fluorescein angiography revealed multiple arterial aneurysms, nonperfused retina, and leakage from neovascularization in the periphery. These findings led to the diagnosis of IRVAN syndrome. We performed vitrectomy with silicon oil tamponade in the right eye, and panretinal photocoagulation to the nonperfused area of the retina in the left eye. However, in the left eye, vitreous hemorrhage recurred. Her serum antinuclear antibody level was high, but no other systemic abnormalities were identified. Conclusion: Abnormal autoimmune status may be associated with the etiology of IRVAN syndrome.