Rituximab Treatment and Long-term Outcome of Patients With Autoimmune Encephalitis
Franziska Thaler, Luise Zimmermann, Stefan Kammermeier, Christine Strippel, Marius Ringelstein, Andrea Kraft, Kurt‐Wolfram Sühs, Jonathan Wickel, Christian Geis, Robert Markewitz, Christian Urbanek, Claudia Sommer, Kathrin Doppler, Loana Penner, Jan Lewerenz, Rosa Rößling, Carsten Finke, Harald Prüß, Nico Melzer, Klaus‐Peter Wandinger, Frank Leypoldt, Tania Kümpfel, Michael Adelmann, Luise Appeltshauser, Ilya Ayzenberg, Carolin Baade‐Büttner, Andreas van Baalen, Sebastian Baatz, Bettina Balint, Sebastian Bauer, Annette Baumgärtner, Sonka Benesch, Robert L. Berger, Sascha Berning, Sarah Bernsen, Christian G. Bien, Corinna Bien, Andreas Binder, Stefan Bittner, Daniel Bittner, Franz Blaes, Astrid Blaschek, Justina Dargvainiene, Andre Dik, Mona Dreesmann, Friedrich Ebinger, Lena Edelhoff, Sven Ehrlich, Katharina Eisenhut, Dominique Endres, Marina Entscheva, Jürgen Faiss, Walid Fazeli, Alexander Finke, Dirk Fitzner, Marina Flotats‐Bastardas, Friedemann Paul, Manuel A. Friese, Marco Gallus, M. M. Gebhard, Christian Geis, Anna Gorsler, Armin Grau, Oliver Grauer, Catharina C. Groß, Halime Gül, Robert Handreka, Niels Hansen, Martin Häusler, Joachim Havla, Ha‐Yeun Chung, Wolfgang Heide, Valentin Held, Kerstin Hellwig, Philip Hillebrand, Frank Hoffmann, Ulrich Hofstadt‐van Oy, Fatme Seval Ismail, Martina Jansen, Max Kaufmann, Christoph Kellinghaus, Susanne Knake, Peter Körtvélyessy, Stjepana Kovac, Markus Krämer, Christos Krogias, Christoph Lehrich, Andreas Linsa, Jan D. Lünemann, Michael P. Malter, Kristin Stefanie Melzer, Til Menge, Sven G. Meuth, Gerd Meyer zu Hörste, Constanze Mönig, Marie‐Luise Mono, Michael Nagel, Tobias Neumann‐Haefelin, Jost Obrocki, Thomas Pfefferkorn
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.