Relapses and Serious Infections in Patients with Neuromyelitis Optica Spectrum Disorder Treated with Rituximab: A Swedish Single-Center Study

Abstract

Neuromyelitis optica spectrum disorder (NMOSD) is a rare immune-mediated relapsing-remitting disease of the central nervous system. The usage of rituximab, as relapse-preventive therapy, in NMOSD is common. We performed a single-center retrospective cohort study to assess the risk of relapses and severe infectious events (SIEs) in rituximab-treated NMOSD patients. This study included 24 aquaporin-4 IgG+ (AQP4+), 8 myelin-oligodendrocyte-protein IgG+ (MOG+), and 10 double-seronegative NMOSD patients. Relapses were observed in 50% of all patients during a mean treatment time of 4.0 (range: 0.5–8.25) years. The incidence risk ratio (IRR) of relapse was three times higher in MOG+ compared to AQP4+ patients (IRR: 3.0, 95% confidence interval (CI); 1.2–7.7). SIEs occurred in 40% of all patients during follow-up. AQP4+ patients conferred an increased risk of SIEs compared to MOG+ patients (IRR; 5.3, 95% CI; 1.2–24.3). Incomplete CD19+ B-lymphocyte suppression was not correlated with relapse risk (hazard ratio; 1.9, 95% CI; 0.7–5.2), and there was no correlation between IgG-levels and SIE risk (odds ratio; 2.0, 95% CI; 0.8–4.8). In conclusion, considerable risks of both relapses and SIEs were observed in NMOSD patients exposed to rituximab, which underlines the need for close clinical vigilance of disease activity and infections during treatment.