A case of refractory idiopathic retinitis, vasculitis, aneurysms, and neuroretinitis (IRVAN) responsive to infliximab and mycophenolate mofetil.

Abstract

Purpose: Idiopathic retinal vasculitis, aneurysms, and neuroretinitis (IRVAN) syndrome is a severe vision-threatening retinal vascular inflammatory disease. As a very rare condition, more reports of cases are needed to better guide diagnosis and management. We report a case of IRVAN initially refractory to adalimumab, rituximab, oral corticosteroids, subtenon triamcinolone injection, and intravitreal bevacizumab therapy that was successfully treated with infliximab and mycophenolate mofetil.

Methods: Case report.

Results: A 24-year-old previously healthy male presented with acute bilateral sequential visual field defects, preceded by one month of photopsias. Examination and imaging revealed bilateral extensive retinal peripheral nonperfusion, vessel pruning, and vessel and disc leakage, consistent with occlusive retinal vasculitis. The additional finding of arterial macroaneurysms was suggestive of IRVAN syndrome. The patient had progressive vessel pruning despite treatment with adalimumab, rituximab, oral corticosteroids, subtenon triamcinolone injection, and intravitreal bevacizumab. Stabilization of his disease with areas of vessel recanalization was achieved with a regimen of infliximab and mycophenolate mofetil, which allowed tapering of oral steroids. Additionally, targeted retinal photocoagulation and intravitreal bevacizumab were required to treat secondary pathologic neovascularization complicated by vitreous hemorrhage.

Conclusions: Idiopathic retinal vasculitis, aneurysms, and neuroretinitis (IRVAN) syndrome is a rare inflammatory condition causing retinal vascular dilatation, exudation, capillary nonperfusion, and sight-threatening neovascularization. We demonstrate a case where infliximab, combined with mycophenolate mofetil and with panretinal photocoagulation to achieve control of neovascular sequelae, was more effective than adalimumab and rituximab at halting progression of disease.