IRVAN (Idiopathic Retinitis, Vasculitis, Aneurysms, and Neuroretinitis) Syndrome:

 IRVAN (Idiopathic Retinitis, Vasculitis, Aneurysms, and Neuroretinitis) Syndrome:

IRVAN is a subtype of retinal vasculitis. It is a rare clinical entity of unknown etiology. Some authors have speculated an association with positive P-ANCAs (Perinuclear AntiNeutrophil Cytoplasmic Antibodies) systemic vasculitis and antiphospholipid syndrome.

Risk Factors

Slight female preponderance. There is no race predilection. Young or middle-aged people mainly affected.

Stages

* 1 : Macroaneurysms, exudation, neuroretinitis, retinal vasculitis.

* Stage 2 : Capillary nonperfusion (angiographic evidence)

* Stage 3 : Posterior segment neovascularization of disc or elsewhere and/or vitreous hemorrhage.

* Stage 4 : Anterior segment neovascularization (rubeosis iridis)

* Stage 5 : Neovascular glaucoma.

Diagnosis:

Three major criteria (retinal vasculitis, aneurysmal dilations at arterial bifurcations, and neuroretinitis) and 3 minor criteria (peripheral capillary nonperfusion,retinal neovascularization, and macular exudation) are used to diagnose IRVAN.

Signs

Multiple postequatorial arterial aneurysms in the retina and optic disc head.

* Peripapillary lipid exudation associated with macroaneurysms and retinal vasculitis.

* Retinal hemorrhages in posterior pole.

* Peripheral capillary nonperfusion

* Retinal vascular sheathing

* Swelling of the optic nerve

* Retinal neovascularization

* Optic disc neovascularization

Symptoms

Asymptomatic

Sometimes a patient will not complaint about a loss of vision in spite of having a severe disease because it is not affected during initial presentation.

Unspecific symptoms

For instance , multiple visits for a change of glasses due to blurred vision.

Symptoms of Retinal Vasculitis.

Diagnostic procedures

Fundus Fluorescein angiography:

FFA highlights the arteriolar abnormalities. The aneurysms are more evident and the alterations in arteriolar caliber are more obvious. Leakage of fluorescein from the aneurysms is apparent. 

Patients with IRVAN usually have optic nerve head staining. Disc and/or retinal neovascularization show marked fluorescein leakage. 

The areas of peripheral non-perfusion are easier to appreciate with FFA, especially wide-field FFA.

Complications

Anterior segment:

Iris neovascularization, Neovascular Glaucoma, blind eye, painful blind eye.

Posterior segment:

Disc neovascularization, retinal neovascularization, vitreous hemorrhage, tractional retinal detachment, retinal artery occlusion.

Management

Currently, retinal laser photocoagulation and the use of intravitreal injections of anti-vascular endothelial grow factor antibodies ( anti-VEGF ) are considered within the first line of treatment.