Treatment of Neuromyelitis Optica with Intravenous Immunoglobulin: Report of 8 Patients (P04.141)

Abstract

Objective: Report the effect of IV immunoglobulin (IVIG) as a rescue therapy for patients with relapsing neuromyelitis optica (NMO) that have failed adequate immunosuppression. Background Treatment of NMO is based on case series that evidence benefit with immunosuppressive drugs (prednisone, azathioprine, micophenolate and rituximab). Plasmapheresis is recommended for patients with severe relapses or that keep on relapsing while on immunosuppressants. Because plasmapheresis is not immediately available in some Brazilian hospitals, requires a central line and the blood derived products, we implemented a program to use IVIG as a rescue therapy for those that kept on relapsing after an adequate immunosuppressant course. Design/Methods: Evaluation 8 patients with NMO that fulfilled the Wingerchuk 2006 criteria, followed at UNIFESP, that first received prednisone (0.5-1mg/kg) and azathioprine (2-3mg/kg) for preventing relapses and further received pulse IV immunoglobulin (IVIG) 2g/kg in five days every three to four months up to 5 years as rescue therapy. Efficacy was evaluated comparing annualized relapse rate (ARR) and progression index (PI) before and after IVIG use. Results: There were seven women and one man with mean age of 30 years and mean disease duration of 7.6 years; five were positive for the NMO-IgG. Reasons for IVIG indication were either severe relapse not improving after 5g IV methylprednisolone, inefficacy to reduce ARR or EDSS worsening. Two to ten cycles of IVIG were implemented to these eight patients; mean ARR decreased from 1.8 (±1.6) to 0.1 (±0.2) and mean PI from 1.5 (±1.6) to 0.9 (±0.6). One patient died due to severe encephalitic and brainstem relapse. Conclusions: IVIG provided additional short and long-term benefits in patients with NMO that have failed immunosuppression or suffered a severe relapse. The study for its use as a rescue therapy in places where plasmapheresis is unavailable should be encouraged based on these 7 patients that presented a positive response. Disclosure: Dr. Bichuetti has nothing to disclose. Dr. Boulos has nothing to disclose. Dr. Shinosaki has nothing to disclose. Dr. Souza has nothing to disclose. Dr. Oliveira has nothing to disclose. Dr. Gabbai has nothing to disclose.