Idiopathic retinal vasculitis, aneurysms and neuroretinitis syndrome associated with positive perinuclear antineutrophil cytoplasmic antibody

Abstract

Purpose: To report a case of idiopathic retinal vasculitis, aneurysms and neuroretinitis (IRVAN) syndrome associated with positive perinuclear antineutrophil cytoplasmic antibody (P-ANCA).

Case report: 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.

Conclusion: IRVAN syndrome may be a retinal component of P-ANCA associated vasculitis.

Keywords: IRVAN Syndrome; P-ANCA.

Figure 1

Fundus photograph of the right eye shows optic atrophy and severe old vascular sheathing.

Figure 2

Fundus photograph of the left eye shows disc edema, hemorrhage and vascular sheathing with macular and perivascular exudation.

Figure 3

Fluorescein angiogram of the right eye reveals diffuse areas of nonperfusion.

Figure 4

Fluorescein angiogram of the left eye reveals numerous aneurysmal dilatations at arterial bifurcation sites, focal areas of delayed choroidal filling and arterial wall staining.