RT Journal Article
SR Electronic
T1 Rituximab Treatment and Long-term Outcome of Patients With Autoimmune Encephalitis
JF Neurology - Neuroimmunology Neuroinflammation
JO Neurol Neuroimmunol Neuroinflamm
FD Lippincott Williams & Wilkins
SP e1088
DO 10.1212/NXI.0000000000001088
VO 8
IS 6
A1 Thaler, Franziska S.
A1 Zimmermann, Luise
A1 Kammermeier, Stefan
A1 Strippel, Christine
A1 Ringelstein, Marius
A1 Kraft, Andrea
A1 Sühs, Kurt-Wolfram
A1 Wickel, Jonathan
A1 Geis, Christian
A1 Markewitz, Robert
A1 Urbanek, Christian
A1 Sommer, Claudia
A1 Doppler, Kathrin
A1 Penner, Loana
A1 Lewerenz, Jan
A1 Rößling, Rosa
A1 Finke, Carsten
A1 Prüss, Harald
A1 Melzer, Nico
A1 Wandinger, Klaus-Peter
A1 Leypoldt, Frank
A1 Kümpfel, Tania
A1 on behalf of the German Network for Research on Autoimmune Encephalitis (GENERATE)
YR 2021
UL http://nn.neurology.org/content/8/6/e1088.abstract
AB 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.abs=antibodies; AE=autoimmune encephalitis; CA=cerebellar ataxia; CASPR2=contactin-associated protein-like-2; CBA=cell-based assay; GAD65=glutamic acid decarboxylase 65; GENERATE=GErman Network for Research on AuToimmune Encephalitis; IHC=immunohistochemistry; IVIG=IV immunoglobulin; LE=limbic/autoimmune encephalitis; LGI1=leucine-rich glioma-inactivated-1; mRS=Modified Rankin Scale; NMDAR=NMDA receptor; RIA=radioimmunoassay; SPS=stiff-person syndrome