Is There a True Neuroretinitis in Idiopathic Retinal Vasculitis, Aneurysms, and Neuroretinitis (IRVAN) Syndrome ?

Purpose: To clarify whether a true neuroretinitis is part of  idiopathic retinal vasculitis, aneurysms, and neuroretinitis (IRVAN) syndrome.

Methods: Critical literature review.

Results: Neuroretinitis has been considered as a prominent feature of IRVAN syndrome. It has been described as late diffuse optic  disc staining on fluorescein angiography. However, patients with IRVAN syndrome usually do not present with visual function alterations consistent with optic neuropathy or neuroretinitis. The typical stellate exudative maculopathy seen in IRVAN syndrome is primarily caused by leakage from retinal arteriolar aneurysms at or near the optic disc, and it could be misinterpreted as a feature of neuroretinitis.

Conclusion: There is no clinical evidence of true neuroretinitis at any of the disease stages in patients with IRVAN syndrome. The acronym « IRVARE » (Idiopathic Retinal Vasculitis, Aneurysms, and Retinal Exudates) is proposed to better characterize this syndrome.