IRVAN Syndrome

An adult patient presented to the Uveitis clinic for evaluation after developing visual deficits in the right eye. He was previously diagnosed with multiple branch retinal artery and vein occlusions in both eyes concerning for an occlusive retinal vasculitis. On examination he was found to have vascular sheathing and narrowing in both eyes. Fluorescein angiography demonstrated diffuse small and large vessel leakage with multiple peripheral arterial occlusions with small arteriolar aneurysms bilaterally. An extensive infectious, inflammatory, cardiac, and hypercoagulability workup was performed with unremarkable results. MRI brain and MRA head was also normal. The patient was diagnosed with IRVAN syndrome and started on high dose oral steroids with improvement in vascular leakage but worsening of the arteriolar aneurysms. He was transitioned to multiple steroid-sparing agents (Azathioprine, Cyclosporine) and given a trial of intravitreal anti-VEGF therapy without adequate disease control. He was started on Adalimumab which resulted in decreased vascular leakage and improvement in macroaneurysms for several years. The decision was made by the patient’s rheumatologist to taper the Adalimumab which resulted in progression of his disease with the development of multiple macroaneurysms. Upon restarting the Adalimumab at its initial dose, the patient’s vasculitis was quiescent, and his vision remained stable 6 years after presentation.

Presentation Date: 03/24/2022

Issue Date: 04/08/2022