Rituximab Treatment and Long-term Outcome of Patients With Autoimmune Encephalitis: Real-world Evidence From the GENERATE Registry.

Thaler FS, Zimmermann L, Kammermeier S, Strippel C, Ringelstein M, Kraft A, Suhs KW, Wickel J, Geis C, Markewitz R, Urbanek C, Sommer C, Doppler K, Penner L, Lewerenz J, Rossling R, Finke C, Pruss H, Melzer N, Wandinger KP, Leypoldt F, Kumpfel T (2021)

Publication Type: Journal article

Publication year: 2021

Journal

Neurology, Neuroimmunology and Neuroinflammation Lippincott, Williams & Wilkins

Book Volume: 8

Journal Issue: 6

DOI: 10.1212/NXI.0000000000001088

Abstract

BACKGROUND AND OBJECTIVES: To determine the real-world use of rituximab in autoimmune encephalitis (AE) and to correlate rituximab treatment with the long-term outcome. METHODS: Patients with NMDA receptor (NMDAR)-AE, leucine-rich glioma-inactivated-1 (LGI1)- AE, contactin-associated protein-like-2 (CASPR2)-AE, or glutamic acid decarboxylase 65 (GAD65) disease from the GErman Network for Research on AuToimmune Encephalitis who had received at least 1 rituximab dose and a control cohort of non-rituximab-treated patients were analyzed retrospectively. RESULTS: Of the 358 patients, 163 (46%) received rituximab (NMDAR-AE: 57%, CASPR2-AE: 44%, LGI1-AE: 43%, and GAD65 disease: 37%). Rituximab treatment was initiated significantly earlier in NMDAR- and LGI1-AE (median: 54 and 155 days from disease onset) compared with CASPR2-AE or GAD65 disease (median: 632 and 1,209 days). Modified Rankin Scale (mRS) scores improved significantly in patients with NMDAR-AE, both with and without rituximab treatment. Although being more severely affected at baseline, rituximab-treated patients with NMDAR-AE more frequently reached independent living (mRS score ≤2) (94% vs 88%). In LGI1-AE, rituximab-treated and nontreated patients improved, whereas in CASPR2-AE, only rituximab-treated patients improved significantly. No improvement was observed in patients with GAD65 disease. A significant reduction of the relapse rate was observed in rituximab-treated patients (5% vs 13%). Detection of NMDAR antibodies was significantly associated with mRS score improvement. A favorable outcome was also observed with early treatment initiation. DISCUSSION: We provide real-world data on immunosuppressive treatments with a focus on rituximab treatment for patients with AE in Germany. We suggest that early and short-term rituximab therapy might be an effective and safe treatment option in most patients with NMDAR-, LGI1-, and CASPR2-AE. CLASS OF EVIDENCE: This study provides Class IV evidence that rituximab is an effective treatment for some types of AE.

Involved external institutions

Ludwig-Maximilians-Universität (LMU) DE Germany (DE) Universitätsklinikum Jena DE Germany (DE) Westfälische Wilhelms-Universität (WWU) Münster DE Germany (DE) Heinrich-Heine-Universität Düsseldorf DE Germany (DE) Martin-Luther-Universität Halle-Wittenberg (MLU) DE Germany (DE) Medizinische Hochschule Hannover (MHH) / Hannover Medical School DE Germany (DE) Universitätsklinikum Schleswig-Holstein (UKSH) DE Germany (DE) Klinikum der Stadt Ludwigshafen am Rhein gGmbH DE Germany (DE) Universitätsklinikum Würzburg DE Germany (DE) Universität Ulm DE Germany (DE) Charité - Universitätsmedizin Berlin DE Germany (DE)

How to cite

APA:

Thaler, F.S., Zimmermann, L., Kammermeier, S., Strippel, C., Ringelstein, M., Kraft, A.,... Kumpfel, T. (2021). Rituximab Treatment and Long-term Outcome of Patients With Autoimmune Encephalitis: Real-world Evidence From the GENERATE Registry. Neurology, Neuroimmunology and Neuroinflammation, 8(6). https://doi.org/10.1212/NXI.0000000000001088

MLA:

Thaler, Franziska S., et al. "Rituximab Treatment and Long-term Outcome of Patients With Autoimmune Encephalitis: Real-world Evidence From the GENERATE Registry." Neurology, Neuroimmunology and Neuroinflammation 8.6 (2021).

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