Journal Article DZNE-2022-00853

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

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2021
Lippincott Williams & Wilkins Philadelphia, Pa.

Neurology: Neuroimmunology & Neuroinflammation ; official journal of the American Academy of Neurology 8(6), e1088 () [10.1212/NXI.0000000000001088]

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Abstract: To determine the real-world use of rituximab in autoimmune encephalitis (AE) and to correlate rituximab treatment with the long-term outcome.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.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.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.This study provides Class IV evidence that rituximab is an effective treatment for some types of AE.

Keyword(s): Adult (MeSH) ; Aged (MeSH) ; Anti-N-Methyl-D-Aspartate Receptor Encephalitis: drug therapy (MeSH) ; Anti-N-Methyl-D-Aspartate Receptor Encephalitis: immunology (MeSH) ; Autoantibodies: immunology (MeSH) ; Autoimmune Diseases of the Nervous System: drug therapy (MeSH) ; Autoimmune Diseases of the Nervous System: immunology (MeSH) ; Encephalitis: drug therapy (MeSH) ; Encephalitis: immunology (MeSH) ; Female (MeSH) ; Follow-Up Studies (MeSH) ; Humans (MeSH) ; Immunosuppressive Agents: administration & dosage (MeSH) ; Immunosuppressive Agents: pharmacology (MeSH) ; Male (MeSH) ; Middle Aged (MeSH) ; Outcome Assessment, Health Care (MeSH) ; Registries (MeSH) ; Rituximab: administration & dosage (MeSH) ; Rituximab: pharmacology (MeSH) ; Autoantibodies ; Immunosuppressive Agents ; anti-CASPR2 autoantibody ; anti-GAD65 autoantibody ; anti-NMDA receptor autoantibody ; anti-leucine-rich glioma-inactivated 1 autoantibody ; Rituximab

Classification:

Contributing Institute(s):
  1. Autoimmune Enzephalopathies (AG Prüß)
Research Program(s):
  1. 353 - Clinical and Health Care Research (POF4-353) (POF4-353)

Appears in the scientific report 2021
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Medline ; Creative Commons Attribution-NonCommercial-NoDerivs CC BY-NC-ND (No Version) ; DOAJ ; OpenAccess ; Article Processing Charges ; Clarivate Analytics Master Journal List ; Current Contents - Clinical Medicine ; DOAJ Seal ; Essential Science Indicators ; Fees ; IF >= 10 ; JCR ; SCOPUS ; Science Citation Index Expanded ; Web of Science Core Collection
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 Record created 2022-05-25, last modified 2023-09-15


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