Successful Use of Adalimumab Monotherapy in Managing Idiopathic Retinal Vasculitis, Aneurysms, and Neuroretinitis (IRVAN) Syndrome

PURPOSE: To report a case of successful control of IRVAN syndrome with adalimumab monotherapy and to describe relapse of aneurysms after dose tapering. METHODS: Case report. RESULTS: A 46-year-old male presented with missing areas in his vision in both eyes and was diagnosed with IRVAN syndrome after comprehensive examination and imaging. The patient was initially treated with a three-month tapering course of high-dose oral prednisone, followed by azathioprine for four months. Due to worsening nonperfusion and vasculitis, cyclosporine was added; however, after five additional months without adequate disease control, both agents were discontinued and biweekly adalimumab monotherapy was initiated. The patient was monitored over five years, including an attempted tapering of the medication. The patient showed marked clinical improvement with adalimumab, including significant reduction in peripheral vascular leakage and aneurysmal dilatations. However, tapering the adalimumab dose from biweekly to monthly led to disease relapse, characterized by increased size and number of retinal aneurysms. Resuming the bi-weekly dose of adalimumab resulted in the regression of aneurysms and improvement in retinal hemorrhage. CONCLUSION: In this single case report, adalimumab monotherapy was associated with control of IRVAN syndrome, including improvement in vascular leakage and aneurysmal dilatations. The recurrence of aneurysms upon dose tapering and improvement after resuming biweekly dosing suggest a possible therapeutic effect on aneurysm control and the potential need for sustained treatment. Further studies are needed to determine the generalizability of these findings.