Abstract
Purpose: To assess the efficacy and safety of rituximab treatment as second-line immunotherapy in pediatric cases of anti-NMDA receptor (NMDAR) encephalitis.Methods: We retrospectively recruited 8 patients with anti-NMDAR encephalitis who were treated with rituximab as second-line immunotherapy. We evaluated the clinical features, laboratory examination results and treatment protocols of the Chinese children and defined good outcomes based on the modified Rankin scale (mRS) score (0–2) at the last follow-up.Results: A total of eight pediatric patients (median age 6.7 years; four female) with refractory anti-NMDAR encephalitis were recruited to the study. Rituximab was given after a median duration of disease of 57 days (range 50.5–113.75 days). The use of rituximab led to a significant reduction in the mRS and CD19+ B-cells compared to before rituximab infusion (P < 0.05). Five patients (62.5%) had a good outcome (mRS ≤ 2) including four patients (50%) who showed complete recovery (mRS = 0) at the last follow-up. Transient infusion adverse events were recorded in 2 patients (25%). Two patients (25%) had severe infectious adverse events (AEs) and two patients with grade 5 (death). None of the patients developed progressive multifocal leukoencephalopathy (PML).Conclusion: Our study provides evidence that rituximab can efficiently improve the clinical symptoms of anti-NMDAR encephalitis in children. However, due to the risk of adverse infections, rituximab should be restricted in pediatric patients with high rates of mortality and disability.
Highlights
The common treatment of autoimmune encephalitis includes surgery and first-line immunotherapy consisting of corticosteroids, IVIg or plasma exchange [1,2,3,4,5]
Safety, and efficacy of rituximab, we extracted data from patients treated for refractory anti-NMDA receptor (NMDAR) encephalitis that were younger than 18 years
All patients met the following inclusion criteria: (a) patients met the diagnostic criteria for anti-NMDAR encephalitis; (b) patients treated with rituximab after they had failed first-line immunotherapy; (c) patients aged between 0 and 18 years; (d) patients met the definition of refractory anti-NMDAR encephalitis
Summary
The common treatment of autoimmune encephalitis includes surgery and first-line immunotherapy consisting of corticosteroids, IVIg or plasma exchange [1,2,3,4,5]. Second-line immunotherapy including cyclophosphamide or rituximab, or in combination is proposed as an effective clinical strategy and can prevent relapse in patients with refractory anti-NMDAR encephalitis [1, 2]. Accumulating evidence has demonstrated the benefit of rituximab in children with CNS diseases including anti-NMDAR. To increase understanding of the benefits and risks of rituximab usage, we conducted a retrospective study of the efficacy and safety of rituximab usage in Chinese children with refractory anti-NMDAR encephalitis
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