RESUMO
To report a case of idiopathic retinal vasculitis, aneurysms and neuroretinitis [IRVAN] syndrome associated with positive perinuclear antineutrophil cytoplasmic antibody [P-ANCA]. A 51-year-old man presented with loss of vision in his right eye since many years ago and blurred vision in his left eye over the past year. Ophthalmologic examination revealed optic atrophy and old vascular sheathing in the right eye and blurred disc margin, macular exudation, flame shaped hemorrhages, retinal vascular sheathing and multiple aneurysms at arterial bifurcation sites in the left eye, findings compatible with IRVAN syndrome. On systemic workup, the only notable finding was P-ANCA positivity. IRVAN syndrome may be a retinal component of P-ANCA associated vasculitis