Long-Term Efficacy of Infliximab Therapy in Idiopathic Retinal Vasculitis, Aneurysms and Neuroretinitis (IRVAN) Syndrome.

PURPOSE: Idiopathic retinal vasculitis, aneurysms, and neuroretinitis (IRVAN) syndrome is a rare inflammatory vision-threatening retinal disease of unknown origin. If untreated, it can lead to severe vision loss from exudative maculopathy and neovascular sequelae of retinal ischaemia. Detailed IRVAN case reporting may help to provide new insights.

METHODS

Successful treatment of a pediatric patient with infliximab.

RESULTS

A 12-year-old girl with IRVAN syndrome received immunosuppressive and laser photocoagulation therapy without improvements. A shift to treatment with infliximab not only reversed disease symptoms on short term, but also led to a complete and long-term cure of the disease. The patient was followed-up for a period of 3 years and infliximab resulted in complete resolution of retinal and optic nerve head aneurysmal dilations, retinal vasculitis and optic disc swelling, and complete resorption of retinal exudation.

CONCLUSION

Infliximab is safe and effective and should be considered as a primary first-line therapy for IRVAN syndrome.